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THE EYE 



AND 



NERVOUS SYSTEM 



THEIR DIAGNOSTIC RELATIONS 
BY VARIOUS AUTHORS 

EDITED BY 

WM. CAMPBELL POSEY, A.B, M.D. 

PROFESSOR OF OPHTHALMOLOGY IN THE PHILADELPHIA POLYCLINIC; SURGEON TO THE WILLS EYE 

HOSPITAL J OPHTHALMOLOGIST TO THE PENNSYLVANIA EPILEPTIC HOSPITAL AND COLONY FARM, AND 

CONSULTING OPHTHALMOLOGIST TO THE STATE HOSPITAL FOR THE INSANE AT NORRISTOWN 

AND 

WILLIAM G. SPILLEK, M.D. 

PROFESSOR OF NEUROPATHOLOGY AND ASSOCIATE PROFESSOR OF NEUROLOGY IN THE UNIVERSITY OF 

PENNSYLVANIA ; CLINICAL PROFESSOR OF NERVOUS DISEASES IN THE WOMAN'S MEDICAL COLLEGE 

OF PENNSYLVANIA ; PROFESSOR OF NERVOUS DISEASES IN THE PHILADELPHIA POLYCLINIC J 

PRESIDENT OF THE AMERICAN NEUROLOGICAL ASSOCIATION (1905) 



ILLUSTRATED 




PHILADELPHIA AND LONDON 

J. B. LIPPINCOTT COMPANY 



LIBRARY of CONGRESS 
Two Cooies Received 

MAY 31 1906 

„_ Copy riff ht Entrv 
CLASS' <Z XXc, No, 
COPY B. 



\ 






Copyright, 1906 

BY 

J. B. Lippincott Company 



ELECTHOTYPED AND PRINTED BY J. B. UPP1NCOTT COMPANY, PHILADELPHIA, U. 8. A, 



CONTRIBUTORS 

¥ 
BURR, C. W., M.D. 
DERCUM, F. X., M.D. 
DUANE, ALEXANDER, M.D. 
FRAZIER, C. H., A.B., M.D. 
HANSELL, HOWARD F., A.M., M.D. 
HIRSCH, WILLIAM, M.D. 
JACKSON, EDWARD, A.M., M.D. 
LLOYD, J. HENDRIE, A.M., M.D. 
MILLS, CHARLES K., M.D. 
POSEY, WM. CAMPBELL, A.B., M.D. 
RISLEY, S. D., A.M., M.D., PH.D. 
SACHS, B., M.D. 
SAILER, JOSEPH, M.D. 
DE SCHWEINITZ, G. E., A.M., M.D. 
SOUTER, WILLIAM NORWOOD, M.D. 
SPILLER, WILLIAM C, M.D. 
TAYLOR, E. W., M.D. 
WALTON, G. L., M.D. 
WEEKS, JOHN E., M.D. 
WEISENBURG, T. H., M.D. 
WOOD, CASEY A., CM., M.D., D.C.L. 
WURDEMANN, H. V., M.D. 



> 



PREFACE 

¥¥ 

Although it is generally conceded that a knowledge of neurology 
is indispensable to the ophthalmologist and that an acquaintanceship 
with ophthalmology is of the greatest service to the neurologist, there 
is no book in the English language which covers the ground where 
these two specialties meet. Several German works and one French 
book have appeared which include much that may be found in these 
pages, but even the student who is conversant with these languages will 
frequently find these books lacking in the information he seeks, and he 
will often be compelled to search through many other sources of infor- 
mation before he obtains what he desires. It has been the aim of the 
editors, therefore, to present under a single cover the phases of ophthal- 
mology and neurology which are in any way connected with each other, 
hoping thereby not only to facilitate the labors of those practising these 
specialties, but also to present ophthalmo-neurology in such a form 
that the general practitioner may without difficulty become ' conversant 
with its symptomatology. 

While it may be impossible for a physician in general practice 
to be at the same time an ophthalmologist and a neurologist, it is quite 
possible, and indeed essential, that he should be aware of the extent, 
as well as of the limitation, of the knowledge of these two specialties 
and of the aid they may offer him in the recognition of disease. So 
many general morbid processes are announced by some minute change 
in the nervous system, and such unusual opportunities of studying 
alterations in the vascular system are presented in the eye, that the 
value of a clear understanding of disease of these structures cannot be 
overestimated, and the necessity for proper training in the methods 
which lead to its recognition, cannot be too strongly insisted upon. 

Realizing the extent of the task before them, and the increased 
value which the work would have if it represented the views of others 
as well as their own, the editors have been most fortunate in securing 
a number of distinguished American ophthalmologists and neurologists 
as collaborators, so that the volume consists of a series of monographs 
by authors peculiarly well fitted by previous study and investigation 
to treat authoritatively of the subjects assigned to them. 



vi PKEFACE 

In dividing the subjects into the various chapters, care was taken 
to avoid repetition of the same matter by different authors. In the 
chapters where overlapping does occur, it was done purposely, with the 
idea of having both the ophthalmological and the neurological phases 
fully presented. 

The editors desire to express their thanks and deep sense of appre- 
ciation to their contributors for the very admirable way in which they 
have treated the subjects allotted to them. It has been a source of 
great regret that the work did not appear, as originally planned, some 
six months ago, but, despite every effort of the publishers, certain labor 
difficulties prevented, and it has been impossible to issue it until now. 

The index has been prepared by Dr. Paul La Eoque and the editors 
would express to him their thanks for his thoroughness. 

The Editors. 
Philadelphia, April 10, 1906. 



TABLE OF CONTENTS 

By William G. Spiller, M.D. 

THE INTRACRANIAL PORTION OF THE ENCEPHALIC NERVES, 

ESPECIALLY THOSE RELATED TO THE EYE. 
The olfactory nerve — The optic nerve — The primary optic centres — " Pupillary 
fibres " in the visual system — The cortical centres of vision — The oculomotor 
nerve — Trochlear nerve — Trigeminal nerve — Abducens nerve — Facial nerve 
— Acoustic nerve — Glossopharyngeal and vagus nerves — Accessorius — Hypo- 
nerve 1 to 19 



CHAPTER II. 

By William Norwood Souter, M.D. 

SIGHT: THE STRUCTURE AND OPTICAL PROPERTIES OF THE EYE. 

The structure of the eye — The eye as a refractory apparatus — Anomalies of 
refraction — Determination of visual acuteness and of the refraction of 
an eye 20 to 56 

CHAPTER III. 

By Charles K. Mills, M.D. 

THE PSYCHOLOGY OF THE VISUAL ACT AND THE FOCAL DISEASES 
OF THE VISUAL CORTEX. 

Introductory outline — The limits and sub-divisions of the visual cortex — His- 
tology of the visual cortex, especially with reference to visual localization — 
The position and the sub-divisions of the primary cortical visual area — The 
focal diseases of the visual cortex — Hemianopias and other defects in the 
visual fields due to cortical lesion — Cerebral chromatic phenomena and the 
cortical centres for color recognition — Amblyopias due to disease of the 
visual cortex or of its entering and connecting tracts — The visual portion 
of the cerebral zone of speech and visual aphasias — Mind blindness — Word 
blindness and allied disorders — Agrammatism — Visual anomia or word 
dumbness 57 to 151 

CHAPTER IV. 
By Casey A. Wood, M.D. 
GENERAL EXAMINATION OF THE EXTERIOR OF THE EYE AND THE 
REGION OF THE EYE ; PERIMETRY ; COLOR-BLINDNESS ; PERIPH- 
ERAL BLINDNESS AND SUBJECTIVE VISUAL SENSATIONS; 
AMBLYOPIA. 

Field of vision — Subjective visual sensations — Amblyopia 152 to 177 

vii 



viii CONTENTS 

CHAPTER V. 

By Alexander Ditane, M.D. 

THE EXTRA-OCULAR MUSCLES. 
Physiology of the eye muscles and eye movements — Tests — Nomenclature and 
classification of the ocular deviations — Paralysis of the individual muscles 
— Spasm of the individual ocular muscles — Convergent and divergent 
anomalies — Comitant heterophoria and squint — Derangements of the asso- 
ciated parallel movements of the eyes — Conjugate paralysis and spasm — 
Tremor of the associated parallel movements, nystagmus and pseudo- 
nystagmus — anomalous deviations of the eyes — Disjunctive movements. 
178 to 284 

CHAPTER VI. 

By John E. Weeks, M.D. 

THE INTRA-OCULAR MUSCLES. 
Iris — Examination of the iris — Light reflexes — Examination of the light 

reflex — Pathological variations in the pupil 285 to 319 

CHAPTER VII. 
By Edward Jackson, A.M., M.D. 

PERIPHERAL AFFECTIONS OF THE FIFTH, SEVENTH, AND CERVICAL 
SYMPATHETIC NERVES; OCULAR LESIONS CAUSED BY THEM: 
TREATMENT. 

The fifth nerve — The seventh nerve — The sympathetic nerve — The secretion and 

removal of tears — The lids and their movements 320 to 359 

CHAPTER VIII. 

BY H. V. WtJRDEMANN, M.D. 

DISEASES OF THE RETINA AND OPTIC NERVE. 
Ophthalmoscopy — Ophthalmic signs of general and nervous diseases — Sensory 
affections — Vascular lesions — Inflammation of the retina — Varieties of 
retinitis associated with general and nervous affections — Inflammation of 
the optic nerve — Optic nerve atrophy 360 to 386 

CHAPTER IX. 
By William O. Spiller, M.D. 

TUMORS AND OTHER LESIONS OF THE BRAIN, ESPECIALLY THOSE 
IN RELATION TO THE VISUAL APPARATUS AND OCULAR 
NERVES. 
General symptoms of tumor — Lesions of the occipital lobe — Tumors of the 
parietal lobe — Tumors of the temporal lobe — Tumors of the motor area — 
Tumors of the frontal lobe — Tumors of the optic thalamus — Tumors of the 
corpora quadrigemina and cerebral peduncles — Lesions of the pineal gland — 
Tumors of the pituitary body — Tumors of the optic nerve — Tumors of the 
pons and medulla — Tumors of the cerebellum 387 to 433 



CONTENTS ix 

CHAPTEK X. 
By E. W. Taylor, M.D. 

BULBAR AND PSEUDOBULBAR DISEASES. 
General remarks — General symptomatology — Progressive bulbar palsy — Acute 
(apoplectiform) bulbar paralysis — Bulbo-pontine hemorrhage — Acute 
bulbar apoplexy — Bulbo-pontine softening — Bulbar paralysis from other 
causes — Superior encephalitis (Wernicke) chronic ophthalmoplegia — 
Pseudo bulbar paralysis — Myasthenia gravis — Paralyzing vertigo (Ger- 
lier's disease. ) 434 to 464 

CHAPTER XL 

By William Hirsch, M.D. 

ARTERIOSCLEROSIS— MULTIPLE SCLEROSIS— PSEUDOSCLEROSIS — DIF- 
FUSE SCLEROSIS— FRIEDREICH'S DISEASE— PARALYSIS AGITANS. 
465 to 477 

CHAPTER XII. 



PARASYPHILITIC AFFECTIONS, INSANITIES, AND TOXIC ENCEPH- 
ALOPATHIES. 

The ocular symptoms of paresis — Summary — The ocular phenomena of tabes — 
Summary — The ocular symptoms in functional insanities — Summary — The 
ocular symptoms of alcoholic intoxication — The ocular symptoms of lead 
intoxication 478 to 531 



CHAPTER XIII. 
By B. Sachs, M.D. 
Amaurotic family idiocy — Infantile cerebral palsies (spastic hemiplegia, 

diplegia and paraplegia ) 532 to 544 

CHAPTER XIY. 
By Charles "W. Burr, M.D. 
Abnormalities in the development of the brain and skull — Progressive facial 
hemiatrophy — Progressive facial hemihypertrophy — Other progressive 
hypertrophies — Acromegaly 545 to 564 

CHAPTER XY. 
By T. H. Weisenburg, M.D. 
DISEASES OF THE SPINAL CORD AND SPINAL NERYES. 
Myelitis — Syringo myelia — Primary degeneration of the pyramidal tracts 
(primary lateral sclerosis) — Amyotrophic lateral sclerosis — Progressive 
ascending unilateral paralysis — Posterolateral sclerosis — Spinal cord symp- 
toms in pernicious anaemia — Injuries of the spinal cord — Acute ascending 
paralysis (Landry's paralysis) — Diseases of the nerves — Multiple neuritis. 
565 to 613 



CONTENTS 



CHAPTER XVI. 
By G. E. de Schweinitz, A.M., M.D. 
NEUROSES AND PSYCHOSES. 
Ocular manifestations of hysteria — Complete anaesthesia of the visual sense, or 
hysterical amaurosis — Incomplete anaesthesia of the visual sense, or hysteri- 
cal amblyopia — Defective and painful visual sensation, or hysterical asthe- 
nopia — Anomalies of accommodation, or hysterical cyclospasm and cyclo- 
plegia — Anomalies of the iris movements or hysterical pupil phenomena — 
Anomalies of the eyelid and eye muscle movements, or hysterical spasms, 
contractures, pseudo-palsies of the palpebral and bulbar musculature — 
Anomalies of convergence — Previously unclassified hysterical ocular phe- 
nomena — Concerning the etiological factors in hysterical ocular stigmata — 
Ocular manifestations of neurasthenia and traumatic neurosis — Treatment 
of the ocular disorders of hysteria and neurasthenia — The ocular manifes- 
tation of hypochondriasis — The ocular manifestation of hypnotism and 
sleep — Tests for detecting pretended amblyopia (malingering) 614 to 696 



CHAPTER XVII. 
By James Hendrie Lloyd, A.M., M.D. 

MIGRAINE, TETANUS, TETANY, EPILEPSY, BLINDNESS DUE TO 
URAEMIA AND OTHER BLOOD STATES. 

Migraine — Ophthalmic migraine — Ophthalmoplegic migraine — Psychical mi- 
graine — Tetanus — Tetany — Epilepsy — Blindness due to uraemia and other 
blood states 697 to 742 



CHAPTER XVIII. 

NEUROSES AND OTHER CONDITIONS OCCASIONED BY ERRORS OF 
REFRACTION AND IMBALANCE OF THE EXTRA-OCULAR 
MUSCLES, AND THE EFFECT OF LENSES AND OPERATIVE PRO- 
CEDURE IN RELIEVING THE SAME. 

Part I. 

By S. D. Risley, M.D. 

Headache ; neuralgia * 743 to 774 

Part II. 
By Howard F. Hansell, A.M., M.D. 

EPILEPSY; CHOREA; VERTIGO; GASTRIC DISTURBANCES ; SPASMODIC 
, MOVEMENTS OF THE HEAD OTHER THAN CHOREIFORM ; GEN- 
ERAL ASTHENOPIA. 
775 to 827 



CHAPTER XIX. 

By Wm. Campbell Posey, A.B., M.D. 
EXOPHTHALMIC GOITRE. 
Symptomatology — The ocular symptoms — Course, prognosis and frequency — 

Pathology — Treatment — Bibliography 828 to 851 



CONTENTS xi 

CHAPTER XX. 
By Wm. Campbell Posey, A.B., M.D. 
THE PSYCHOLOGICAL EFFECTS OF OPERATIONS ON THE EYES. 
The relief of insanity by operations upon the eyes — Mental disturbances after 

operation upon the eyes — Bibliography 852 to 866 

CHAPTER XXI. 

By Charles H. Frazier, M.D. 

THE SURGICAL TREATMENT OF INTRACRANIAL LESIONS CAUSING 

DISTURBANCES OF VISION. 
Lesions of the cranial sinuses as affecting the contents of the orbit — Thrombosis 
of the cavernous sinus — Tumors of the occipital lobe — Preservation of vision 
in cases of brain tumors — Tumors of the chiasm — Operations upon the cere- 
bellum for the relief of choked disks — Surgery of the seventh nerve — Opera- 
tive treatment of facial palsy — Visual disturbance due to internal hydro- 
cephalus — Surgery of the fifth nerve — Trifacial neuralgia, ticdouloureux. 
867 to 905 

CHAPTER XXII. 
By Joseph Sailer, M.D. 
TREMORS, REFLEXES, GAITS. 
Tremors — Classification — Reflexes — Reflexes of the head — Reflexes of the upper 
extremity — Reflexes of the lower extremity — Reflexes of the trunk — The 
literature on reflexes — Gaits — The gait as a symptom of nervous disease — 
Methods of study — The paralytic gaits — Gaits due to disturbance of co- 
ordination — The gaits of functional nervous disease — Delayed walking. 
906 to 950 

CHAPTER XXIII. 

By G. L. Walton, M.D. 

DEGENERACY (DEGENERATION, CONSTITUTIONAL DEFECTS, 

DEVIATION). 
General considerations — The bearing of constitutional deviation upon the esti- 
mation of eye strain symptoms — Deviation — The bibliographical study of 
constitutional deviations — Anatomical stigmata — Physiological stigmata — 
Psychic and psycho neurotic stigmata 951 to 971 



THE 

EYE AND NERVOUS SYSTEM 



CHAPTEE I. 

THE IKTEACEAOTAL POETIOX OF THE ENCEPHALIC 
JEEVES, ESPECIALLY OF THOSE EELATED TO THE 
EYE. 

By WILLIAM G. SPILLER, M.D. 

THE OLFACTORY NERVE. 

The olfactory, or first, nerve has its origin in the regio olf actoria of 
the Schneiderian membrane. After the fibres constituting this nerve 
have entered the cranium through the cribriform plate, they penetrate 
the olfactory bulb and terminate in the olfactory glomeruli. The 
olfactory nerve, therefore, has a very short intracranial course, its intra- 
cranial connections belonging to the very complex central system of 
fibres of the olfactory apparatus. 

THE OPTIC NERVE. 

The optic, or second, nerve arises and terminates within the retina, 
and the structure commonly called the optic nerve is in reality a part 
of the brain. 

Decussation of the Visual Fibres in the Optic Chiasm. — Were it not for 
the opinion of Kolliker, probably there would not have been so much 
doubt concerning the decussation in man of the nerve-fibres in the optic 
chiasm. Kolliker believes that the decussation is complete, and his 
views have justly received much critical attention. The subject has 
been presented clearly by Warrington and Dutton in a contribution by 
which they try to show that Kolliker is in error, and in this attempt 
they are aided by the investigations of many others. Kolliker admits 
the existence of a small number of direct fibres which pass to the tract of 

1 



2 THE EYE AND NEKVOUS SYSTEM 

the same side as the optic nerve from which they are derived. He is not 
alone in his views because, according to Warrington and Dutton, Biesia- 
decki in 1861, Mandelstamm in 1873, Scheel, W. Krause in 1897, and 
Michel, 1873 to 1890, also have favored the view that the decussation 
is complete. Michel's argument that if homonymous bilateral hemi- 
anopsia is to be explained by the decussation in the chiasm, the pro- 
portion of crossed to uncrossed fibres should be as 1 : 1, and not, as it is 
admitted to be by the adherents of a partial crossing, as 3 : 1, or 4 : 1, or 
even as 5 : 3, is answered by Warrington and Dutton. In a case studied 
by them there was not any marked difference between the amount of 
degeneration in the two optic tracts, although only one optic nerve was 
diseased. Furthermore, they point out, that owing to the limitation 
of the field of vision, the outer and lower parts of the retina are prob- 
ably less developed than the inner and lower, and, as Michel himself 
has found, the number of fibres passing to different sections of the retina 
is very variable, being least in the outer segment. They refer to 
Michel's statement that no anatomical evidence has yet been produced 
demonstrating the double representation of the macula in the cerebral 
cortex, which is required by the theory of semi-decussation in order to 
explain the freedom of the fixation point in cases of hemianopsia, and 
they remark that Michel's hypothesis cannot be regarded as giving a 
more satisfactory explanation. They refer also to Griitzner's work on 
this subject. This investigator formed models of the chiasm in which 
one-half of the fibres were made to cross and the other half to remain on 
the same side. The models were then embedded in paraffin and cut 
into a series of horizontal sections. From a study of these sections 
Griitzner concluded that if he had not known that the decussation was 
partial he would have supposed it to be complete. 

Warrington and Dutton's case was one in which enucleation of the 
right eyeball had been performed on account of trauma. The chiasm 
with the optic nerves attached was cut in a complete series of about 500 
horizontal sections. In the sections about the median plane the atrophy 
from the right nerve was seen spreading in the central portion of the 
same-sided tract, and occupying the front and back of the chiasm, its 
extent being larger in the latter situation. Even though Michel has 
shown that the fibres of the optic nerve, as they enter the chiasm, turn 
sharply at a right angle, and again at another right angle when entering 
the tract of the opposite side, Warrington and Dutton believe they were 
able to detect groups of fibres passing along the outer border of the 
nerve and into the tract of the same side; and they state that all ob- 
servers (Purtscher, Wieting, Siemerling, Williamson) with the modern 



THE ENCEPHALIC NERVES 3 

methods, with the single exception of Michel, are of the opinion that 
degeneration occurs in both optic tracts after destruction of one optic 
nerve ; and the crossed fibres usually occupy the centre of the tract, the 
uncrossed the periphery. 1 

The findings that Probst obtained in his experiments on the optic 
fibres resemble very closely those of von Monakow. Probst believes that 
the decussation of the optic fibres in the dog and cat is a partial one. 
The medial-central fibres of the optic nerve cross first, and later the 
dorsal and ventral fibres, while the lateral fibres do not decussate. 
Most of the optic fibres cross to the opposite optic tract. 

When an eyeball is removed degenerated optic fibres are found in 
both external geniculate bodies, but more are found in the opposite 
ganglion. 

The arm of the anterior colliculus of the corpora quadrigemina 
contains chiefly crossed, but also uncrossed, optic fibres. 

In studying the optic chiasm of a new-born child, stained by the 
hematoxylin method of Weigert, a partial decussation seems to me very 
probable. The course of the nerve-fibres in the optic nerves, chiasm, and 
optic tracts may be better seen in specimens obtained from a new-born 
child than in those from an adult, because the number of stained fibres 
is less in the former. A careful study of the literature on decussation 
of the optic fibres seems to show that the weight of evidence is in favor 
of a partial decussation, and in support of this view are the important 
studies of Ramon y Cajal. 

Parsons, from his investigations on monkeys, concludes that, speak- 
ing generally, the fibres derived from various parts of the retina retain 
their relative position in the optic nerve, — i.e., temporal fibres remain 
external, nasal internal. The temporal fibres tend towards the dorsal 
side as they approach the chiasm, and the nasal towards the ventral 
side. A further dislocation, affecting the temporal fibres most, occurs 
as the result of the intercalation of the papillomacular bundle. There 
may be some variation in different individuals. 2 

According to Henschen, as given by Wilbrand and Saenger, the 
uncrossed bundle (not papillomacular fibres) near the eyeball is divided 
into two parts, between which are placed the fibres of the papillomacular 
bundle, more centrally the two portions of the uncrossed bundle ap- 
proach one another and in the posterior half of the optic nerve in trans- 
verse section unite and assume a sickle shape and are situated in the 

1 Warrington and Dutton, Brain, 1900, vol. xxiii., No. 92, p. 642. 

2 Lancet. April 16, 1904. p. 1029. 



4 THE EYE AND NEKVOUS SYSTEM 

ventrolateral portion of the nerve. The crossed bundle (not papillo- 
macular bundle) has a dorso-medial position throughout the optic nerve. 

The structure of the chiasm is very complicated. Probably the most 
important fact is that the uncrossed fibres are mingled with the crossed 
in the lateral portion of the chiasm, so that nasal hemianopsia can not 
be caused by a lesion of the chiasm. 

There is much difference of opinion, as shown by Wilbrand and 
Saenger, regarding the existence of a separate bundle of uncrossed fibres 
in the optic tract, some believing that the crossed are mingled with the 
uncrossed fibres. Henschen is of the opinion that the uncrossed bundle 
occupies the superior and external position and is distinct from the 
crossed fibres. 

Decussation of the Macular Fibres. — Dean and Usher have attempted 
to determine the position of the optic nerve-fibres in monkeys. Vossius, 
in a case of symmetrical central scotoma in man, found that the macular 
fibres lie close under the dorsal margin of the chiasm. One of the cases 
reported by Dean and Usher corresponds closely with Vossius's descrip- 
tion. Bunge found, they say, that the papillomacular bundle main- 
tains a dorsal position throughout the chiasm in man. Dean and Usher 
give the course of the macular fibres in the chiasm in the monkey 
as follows : Anteriorly in the chiasm they occupy the central area of 
both halves. Soon after entering the chiasm they pass towards the 
dorsum before any crossing has occurred. The crossing takes place 
first at the dorsal periphery, at some distance behind the front of the 
chiasm. On proceeding backward, though still partly remaining dorsal, 
they spread from the dorsal surface in a ventral direction. The macular 
fibres never reach the ventral periphery. These investigators were 
unable to determine whether the macular fibres cross wholly or par- 
tially in the chiasm, as they could not be certain whether the wound 
was limited to the macular fibres, or whether it had damaged only a 
portion of these. 3 

According to Henschen, as quoted by von Monakow, the bundle from 
the macula lutea between the retina and the primary optic centres lies 
in the middle of the transverse sections of the optic nerve and optic 
tract, whereas the rest of the retina is represented in the periphery of 
these structures. 

Wilbrand and Saenger describe the course of the papillomacular 
bundle according to Uhthoff as follows : In the forward portion of the 
optic nerve the bundle lies in wedge form in the outer and lower part 

3 Dean and Usher, Brain, 1903, vol. xxvi., No. 104, p. 524. 



Fig. 1.— Case of complete absence of the visual system; other parts of the body, except the 
external genitalia, were well developed. The age of this patient was twenty-two years. The limbs 
were spastic. 




Fig. 2.— Base of the brain from the case of complete absence of the visual system. There is no 
indication of optic tracts, chiasm, or optic nerves. 




Fig. 3.— Portion of the brain from the case of complete absence of the visual system. A part of the 
right temporal lobe has been cut away, in order to show the absence of the optic tract and external 
geniculate body. The internal geniculate body is well developed. 



l '" 




Fig. 4— Portion of the brain from a case of complete absence of the visual system. The pul- 
vinar is of good size on each side. The anterior colliculi of the corpora quadrigemina appear smaller 
than the posterior, because they were further from the camera. 



THE ENCEPHALIC KEEVES 5 

of the nerve, with the point of the wedge toward the central vessels, and 
from here posteriorly gradually assumes a crescent shape, the ends of 
the crescent withdrawing from the periphery. After the exit of the 
central vessels from the optic nerve the bundle gradually assumes a 
vertically oval form, and becomes centrally placed in the posterior or- 
bital part of the nerve. There are variations in different cases as 
regards the point at which the bundle becomes central. The bundle is 
central also in the intracranial part of the optic nerve, but because in 
this portion the nerve is more oval the bundle has a greater transverse 
than a vertical diameter. In the tract the bundle occupies the centre. 

PRIMARY OPTIC CENTRES. 

The fibres of the optic nerve end chiefly in the external geniculate 
body, but some pass to the posterior part of the optic thalamus known 
as the pulvinar and to the anterior colliculus of the corpora quadri- 
gemina. The fibres to the latter structure in man cannot be numerous, 
although they are in the rabbit. In a case studied by me in which 
the visual system was absent in an adult there was no external geniculate 
body and no trace of either optic nerve ; the anterior colliculi of the 
corpora quadrigemina and the pulvinar on each side were well formed. 

The external geniculate body, the anterior colliculus of the corpora 
quadrigemina, and the pulvinar are known as the primary optic centres, 
and from these bodies other fibres pass to the visual cortex of the brain, 
although they contain also the ramifications of fibres that arise in the 
visual cortex (Edinger). The optic tract is formed chiefly by nerve- 
fibres that terminate in the primary visual centres, but according to 
Edinger they contain also, certainly in the rabbit and cat and probably 
in man, fibres that arise in the corpora quadrigemina; These fibres from 
the primary visual centres terminate in the retina. According tovonMon- 
akow as much as 80 per cent, of the visual fibres in man terminate in 
the external geniculate body, and all the visual fibres of the optic tract 
terminate in the primary visual centres. Yon Monakow is authority 
for the statement that hemianopsia as the result of a lesion confined to 
the anterior colliculus of the corpora quadrigemina has not been ob- 
served, and it is doubtful whether a lesion confined to the pulvinar and 
not implicating the external geniculate body causes hemianopsia. 

Some optic fibres pass above and below the external geniculate body 
as stratum zonale to the pulvinar of the optic thalamus and near to the 
ganglion habenula?, with which, however, they do not come in contact 
(Probst). 4 

4 Probst, Monatsschrift fur Psychiatrie und Neurologic. September, 1900, vol. 
viii. p. 165. 



6 THE EYE AND NERVOUS SYSTEM 

"PUPILLARY FIBRES" IN THE VISUAL SYSTEM. 

Von Gudden showed that the optic nerve contains thick and slender 
nerve-fibres, and, according to von Monakow, the former pass almost 
entirely to the anterior colliculi of the corpora qnadrigemina. These 
thicker fibres are supposed to serve a special function, — viz., the contrac- 
tion of the iris in the light reflex. They have been recently studied by 
Reichardt. It is remarkable that these " pupillary fibres" should be 
present in the optic nerve and optic tract, and although they have not 
been actually demonstrated much evidence of their existence has been 
collected. They were first recognized by von Gudden and von Bech- 
terew, and are believed to have no part in vision. Their function is to 
convey centripetal impulses to the pupillary reflex centre wherever that 
is located. The results obtained by Massaut in his study of the 
" pupillary fibres" are so extraordinary that they may be referred to 
in this connection. This investigator extirpated the iris in rabbits, and 
found degenerated fibres by the Marchi method in the optic nerve, 
which he regarded as " pupillary fibres." The interpretation of this 
degeneration offered by him is accepted with some difficulty, inasmuch 
as a degeneration of one group of fibres, the centripetal, is supposed to 
have occurred as a result of a lesion of the distal ends of another group 
of fibres, the centrifugal. This, in my experience, is so unlike what 
occurs elsewhere in the nervous system that I can accept Massaut 's 
interpretation only with much hesitation, especially as it is well 
known that the Marchi method is at times the source of many erroneous 
conclusions. (See chapter vi., p. 291.) 

Westphal found that the large fibres in the optic nerve receive their 
medullary sheaths sooner than the fine fibres, and he also concluded that 
the former are " pupillary fibres." Reichardt points out that disease 
of the optic nerve may cause complete blindness and yet leave the iritic 
reflex intact, and this seems to indicate that there are distinct systems 
of fibres in the optic nerve. In compression of the optic nerve the iritic 
reflex is much less affected than vision, which may indicate that the 
" pupillary fibres" are more resistant. When acute inflammation of the 
optic nerve is disappearing improvement is usually noticed in the reac- 
tion of the iris before vision begins to return. 

Piltz describes a case in which blindness had existed nine years 
and was caused by optic atrophy, and yet the iritic response to light was 
preserved. Bach observed a case of tabes in which the reflex action of 
the iris to light was better in the eye in which vision was more affected 
and the optic nerve atrophy was more advanced. All this seems to indi- 
cate that there are probably special fibres in the optic nerve for the 



THE ENCEPHALIC NERVES 7 

iritic reflex. Reickardt also has observed an important case. A man 
had atrophy of the left optic nerve, and until four weeks before the 
loss of sight in the left eye became complete he had normal pupillary 
reaction, and even later the reaction was only somewhat .tardy. The 
left optic nerve was found to be small, and the reduction in size was 
caused chiefly by the degeneration of the fine fibres. This case seems 
to afford some support for the widely accepted teaching that the fine 
fibres especially are concerned with vision. 

Reichardt believes that the " pupillary fibres" of the optic nerve may 
alone degenerate, or at least be more affected, and in such a case vision 
would be preserved although the iritic reflex would be lost. 

According to Bach and Meyer, it is probable that the pupillary reflex 
may be stimulated from any part of the retina, although the macula 
region is probably the most important. The " pupillary fibres" are 
mingled with the visual fibres in the optic nerve, although Bach be- 
lieves a certain order is preserved, so that those fibres that come from 
the temporal side of the retina remain in the temporal side of the optic 
nerve. In man the decussation of the " pupillary fibres," as is that of 
the visual fibres, is partial, although most of the fibres of each variety 
cross. The visual and " pupillary" fibres are associated in the optic 
tract as far as the external geniculate body, but here they separate. 

It may be said that no one has ever been able to trace the connec- 
tion between the optic nerve and the oculomotor nucleus. It has been 
supposed that this connection is made by means of Meynert's fibres 
passing in a curve below the aqueduct of Sylvius. Some authors have 
accepted this view as an established fact, forgetting or being in igno- 
rance of the uncertainty concerning this subject. No one disputes that 
these fibres of Meynert from their position might serve as the connect- 
ing link, but there are other fibres in this region which might have this 
function. 

Probst, whose anatomical studies of the nervous system are so well 
known, says that when an eyeball has been enucleated degenerated fibres 
of the optic nerve cannot be followed to the oculomotor nucleus. The 
connection of the optic fibres with the oculomotor nucleus must there- 
fore be by means of a short system of fibres (Schaltsystem). 

It is supposed that the " pupillary fibres" pass from the optic tract 
to the corpora quadrigemina through the arm of the anterior colliculus 
of this body, but the further course of these fibres is unknown. Bern- 
heimer believes he has been able to trace these fibres to the region 
below the aqueduct of Sylvius and near to the oculomotor nucleus, but 
Dimmer, Bach, and others have followed the fibres onlv so far as the 



8 THE EYE AND NERVOUS SYSTEM 

arm and the upper part of the anterior colliculus. The ' ' pupillary fibres" 
are believed by some to be in relation with the stratum zonale of the 
pulvinar of the optic thalamus, with the corpus hypothalamicum, and 
with the ganglion habenulse, but this relation seems improbable. 

Edinger, in the latest edition of his lectures upon the anatomy of 
the nervous system, expresses himself as in doubt concerning the con- 
nection of the " pupillary fibres" of the optic nerve with the oculomotor 
nerve. While acknowledging that the " pupillary fibres" probably pass 
through the anterior arm of the corpora quadrigemina, he is uncertain 
concerning the manner of their connection with the oculomotor nerve. 
Von Monakow also is cautious concerning this supposed connection. 

THE CORTICAL CENTRES OF VISION. 

One of the earliest cases, if not the earliest case, of bilateral hemi- 
anopsia from a unilateral lesion was that published by Lewick ; 3 the 
lesion was an abscess of the right occipital lobe. 

Henschen believes that only a lesion of the cortex of the calcarine 
fissure, and especially of its anterior two-thirds, causes persistent bilat- 
eral homonymous hemianopsia. Lesions of other parts of the cortex of 
the occipital lobe may exist without such visual disturbance. Von 
Monakow says that Huguenin assumed that destruction of the cortex of 
the calcarine fissure causes hemianopsia. Later investigators extended 
the visual cortex ; von Monakow at first included the cuneus, lingual 
lobule, and descending gyrus. Seguin limited the visual cortex to the 
cuneus; Nothnagel limited it to the cuneus and first occipital gyrus; 
Vialet to the entire medial part of the occipital lobe ; Ferrier, Ange- 
lucci, Bianchi, Reinhard, Luciani, Seppilli included the cuneus and 
the medial part of the occipital lobe ; the first extended it to the third 
occipital gyrus and even the angular gyrus. 

Henschen says that all the lateral surface of the occipital lobe and 
the angular gyrus may be destroyed without causing hemianopsia. In 
one of his cases a lesion was confined to the calcarine cortex, and yet 
hemianopsia was caused by this lesion. In another case the calcarine 
cortex was destroyed and hemianopsia was caused thereby, while ex- 
tensive destruction of the lateral surface of the other hemisphere, pre- 
sumably the occipital lobe is meant, did not cause hemianopsia. The 
cases Henschen refers to seem to show that anopsia in the lower quad- 
rant may be caused by a lesion of the dorsal part of the external 
geniculate body, and that in such cases normal fibres may be traced 



5 Amer. Jour, of the Med. Sci., 18GG, 



THE ENCEPHALIC NERVES 9 

from the lower part of the external geniculate body to the inferior lip 
of the calcarine fissure ; by means of these fibres are conveyed the 
impressions made on the inferior part of the retina. His observations 
seem to show that the fibres of the superior lip of the calcarine fissure 
are in connection with the superior part of the retina. Henschen com- 
bats Eerrier, who places the visual centre in the angular gyrus, and 
Nothnagel and Munk, who regard the first occipital convolution as 
part of the visual centre. He has observed anopsia of the superior 
quadrant caused by softening of the inferior lip of the calcarine fissure. 
In another case horizontal scotoma was caused by a lesion confined to 
the base of the calcarine fissure. 

These investigations of Henschen are extremely important, and 
seem to show that the upper part of the retina is represented in the 
upper part of the external geniculate body and in the upper lip of the 
calcarine fissure ; that the lower part of the retina is represented in the 
lower part of the external geniculate body and in the lower lip of the 
calcarine fissure; and that the horizontal zone of the retina, midway 
between the upper and lower portions, is represented in the base of the 
calcarine fissure. Erom this it follows that the fibres from the different 
portions of the retina in the optic nerve and optic tract must preserve 
their relative positions. 6 

The case of quadrant anopsia, recently reported by Beevor and 
Collier (Brain, 1904), with necropsy and careful microscopical study, 
is exceedingly valuable in determining the location of the cortical lesion 
causing quadrant anopsia. A case similar to this has been reported 
by Hun. The only symptom of nervous disease presented while Beevor 
and Collier's patient was under observation was blindness of the left 
upper quadrants of both visual fields, the fixation point escaping. This 
condition remained constant nearly two years. An occlusive lesion of 
the right posterior calcarine artery had caused destruction of the cor- 
tex (1) of the right fusiform lobe for its posterior two centimetres, (2) 
of the right lingual lobe from the junction of the calcarine and parieto- 
occipital fissures to the pole of the hemisphere, (3) of the whole cortex 
in the depth of the calcarine fissure, (4) of the greater part of the 
inferior cuneal gyrus, small areas only at the anterior and posterior 
limits of this gyrus being free. The necrosis did not involve the optic 
radiations at any point. The only parts of the cortex of the mesial 
aspect of the occipital lobe which had escaped destruction were the 
upper two-thirds of the cuneus and the anterior and ventral portion of 



"Henschen, Somaine Medioale. April 22. 1903, No. 10. p. 12,"). 



10 THE EYE AND NEKYOUS SYSTEM 

the fusiform gyrus. The lower quadrants of the visual fields were 
entirely unaffected. The case seems to show that the cortex of the upper 
two-thirds of the cuneus is the visual centre for the lower quadrants. 

In this case the cortex lining the calcarine fissure was completely 
necrotic. The primary half-vision centre, the authors think, cannot 
therefore be limited to the calcarine cortex, nor the macula exclusively 
represented in the anterior part of this region. The sharp distinctions 
of the upper and lower fields as related to the calcarine fissure, made 
by Henschen would seem by this case to need some modification, for the 
lower part of the retina would appear to be represented in the upper 
lip of the calcarine fissure. 

Harris 7 thinks it is possible to have a loss of the lower quadrant on 
the one side and of the upper quadrant on the other from a lesion in 
each cuneus, and he says such a case has been recorded by Weymann, 
but how the function of only one quadrant of the retina of one eye can 
be lost by a lesion of the visual cortex is to me unintelligible. Even 
granting that the crossed and uncrossed fibres remain separate so far as 
the optic tract, we have no proof that they are ununited in the optic 
radiations and visual cortex. A lesion in these parts seems to cause 
disturbance of vision in each eye. 

Wilbrand thinks that every part of the macula is represented both 
in the right and left visual cortical areas, but in cases in which the 
dividing line in hemianopsia passes directly through the fixation point 
the double representation does not occur. It has also been suggested 
(Forster) that the macula region of the cortex is better supplied with 
blood-vessels than other parts of the visual cortex, and receives nour- 
ishment through the posterior cerebral artery and the occipital branch 
of the artery of the Sylvian fossa. Yon Monakow thinks that the rep- 
resentation of the macula is not confined to the middle of the visual 
centre, nor to its periphery, but he believes that all parts of the occipital 
lobe and possibly even the posterior part of the angular gyrus are repre- 
sented in the macular field, and that the macular field may extend far 
beyond the limit of the area usually recognized as the visual region of 
the cortex. He does not accept Henschen' s views that the macula is 
represented in the anterior portion of the cortex of the calcarine fissure. 

Yon Monakow says that a careful examination of the reported cases 
shows that hemianopsia is most likely to occur and to be persisting per- 
haps only from a lesion in the calcarine fissure, but there are some 
cases in which hemianopsia has occurred from lesion elsewhere, 

7 Brain, vol. xx., 1897, p. 308. 



THE ENCEPHALIC XERVES 11 

although, it may be possible that in these cases the hemianopsia was 
caused by implication of the optic radiations, but the same is true when 
the lesion is in the calcarine fissure. Yon Monakow thinks that in 
every persisting cortical hemianopsia there is partial injury of some 
part of the occipital lobe and — which is more important — also a lesion 
of the optic radiations. 8 

Eew anatomists place the visual centre in the angular gyrus, but 
there is more doubt as to the implication of the lateral surface of the 
occipital lobe in the visual field, and as to the extent of the surface 
involved. 

Gallemaerts has found that after enucleation or atrophy of an eye- 
ball the cells of the visual cortex become less numerous, and this is to 
be seen in both occipital lobes after a lesion of one eyeball. This find- 
ing confirms the partial decussation at the optic chiasm. The crossed 
fasciculus contains more fibres than the direct fasciculus ; therefore the 
greatest diminution of nerve cells is in the visual cortex opposite to the 
side on which the eyeball has been lost. 9 

From a study of the diminution in the number of nerve-cells Galle- 
maerts concludes that the fusiform lobule, the angular gyrus, and the 
descending gyrus (which is on the outer surface of the occipital lobe 
and at the occipital point) are not part of the visual centre, but that the 
visual centre consists of the lingual lobule and the cuneus. In these 
convolutions, especially near the calcarine fissure, the lesions were very 
distinct. The diminution of the nerve-cells in the visual cortex after 
destruction of the eyeball occurs only when years have passed since the 
lesion developed, and all the layers of nerve-cells in the visual cortex 
are affected after a sufficiently long period. 

Bernheimer includes the cuneus, the cortex of the calcarine fissure, 
the lingual lobe, and the descending gyrus in the visual cortex. 

The reader is referred to the article by Dr. Charles K. Mills for 
further remarks on the lower and higher cortical visual areas. 

THE OCULOMOTOR NERVE. 

The nucleus of the oculomotor, or third, nerve lies below the aque- 
duct of Sylvius. This group of cells consists of a median nucleus with 
a lateral nucleus on each side of it. Anterior to these a group of small 
cells is found on each side of the median line, and is known as the 



8 Yon Monakow, Gehirnpathologie. 

9 Gallemaerts, Bulletin de l'Aeademie Royale de Medecine de Belgique, 1902. 
iv. series, vol. xvi. p. 267. 



12 THE EYE AND NEKVOUS SYSTEM 

Edinger-Westphal nucleus. It has been considered as the source of 
the innervation of the inner muscles of the eyeballs, but there is doubt 
whether it should be regarded as a part of the oculomotor nucleus. The 
fibres of the oculomotor nerve pass ventrally through the tegmentum of 
the cerebral peduncle and reach the surface at the inner border of the 
foot of the cerebral peduncle (crusta). The individual fibres forming 
this nerve are not closely united even at the exit of the nerve from the 
cerebral peduncle, and it is because of this that paralysis of one or 
more of the muscles innervated by the oculomotor nerve may be caused 
by basal meningitis without paralysis of the whole muscular supply of 
this nerve, and such paralysis may mistakenly be attributed to nuclear 
disease. 

There is some doubt whether the muscles of the eye innervated by 
the oculomotor nerve are separately represented in the nucleus of this 
nerve. The knowledge concerning this subject that has been obtained 
depends chiefly on investigations upon monkeys. Siemerling believes 
that the investigations of Bach, Bernheimer, van Gehuchten, and van 
Biervliet have not led to uniform results. The oculomotor nucleus, 
according to Siemerling, 10 is merely the place for the transference of 
impulses, and we are not in a position to state which parts are concerned 
with the innervation of the individual muscles. Edinger's views as 
contained in the seventh edition of his lectures upon the structure of the 
nervous system are as follows : The most frontal group supplies the 
levator palpebrse and rectus superior of the same side. From the caudal 
portion arise the fibres for the rectus inferior and the trochlearis of 
the opposite side. The intervening cells give origin to both direct and 
crossed axis cylinder processes, so that each obliquus inferior and each 
rectus internus receives fibres from the right and left lateral group of 
cells. The nerve-cells for the rectus internus are on each side so near 
the large cell median nucleus that Edinger believes the three groups 
together may form a centre for convergence. All the external ocular 
muscles he believes are supplied from the nuclei of the oculomotorius, 
trochlearis and abducens. The fibres for the inner ocular muscles (iris 
and ciliary muscle) arise in the ciliary ganglion, and fine oculomotor 
fibres terminate in this ganglion ; possibly these fibres arise in the 
small cell frontal groups. 

Edinger says it is not certain whether fibres pass directly from the 
oculomotor nucleus to the iris, or whether they are interrupted in the 
ciliary ganglion ; the latter seems the more probable. Bernheimer has 



Archiv firr Psyehiatrie, vol. xl., No. 4, p. 61. 



THE ENCEPHALIC NERVES 13 

caused paralysis of the iris in the monkey by a lesion of the frontal 
portion of the oculomotor nucleus. 

The connection of the cortical centres with the nuclei of the ocular 
nerves has not yet been determined, and the location of the cortical cen- 
tres is still very uncertain. 

Marina's investigations have made it doubtful whether a centre for 
convergence in the oculomotor nucleus exists. He has shown that when 
another ocular muscle was transplanted and allowed to unite with the 
tendon of the internal rectus, contraction of the pupil in convergence 
still occurred, although the internal rectus muscle had no part in the 
convergence movement of the eyeball. Contraction of the pupil oc- 
curred when the eyeball was drawn inward through electrical irritation 
of the internal rectus, and even when it was drawn inward by forceps 
without muscular action. All these experiments seem to show that 
there can be no centre for convergence. 11 

Van Gehuchten and van Biervliet, from their studies on rabbits, 
conclude that the uncrossed fibres of the oculomotor nerve arise chiefly 
in the ventral portion of the nucleus and the crossed fibres in the dorsal 
part of the nucleus. The upper end of the nucleus, therefore, gives 
origin only to direct fibres. The nerve-cells scattered between the fibres 
of the posterior longitudinal bundle (the lateral cells of Bernheimer, 
the aberrant cells of Biervliet) belong to the oculomotor nucleus and 
give origin to direct fibres. 

Bernheimer 12 believes that the muscles of the eyeballs are in- 
nervated from the lateral nucleus (chief nucleus) and that the median 
nucleus and the Edinger-Westphal nucleus innervate only the inner 
muscles of the eye. 

The middle portion of the lateral nucleus, which gives origin to 
crossed fibres, according to this investigator, innervates the inferior 
rectus : further forward is the nucleus of the internal rectus of the 
same side. The lateral portion of the nucleus gives origin to 
fibres for the internal rectus. The group of cells innervating the 
superior rectus is further forward than that innervating the internal 
rectus. The frontal portion of the lateral nucleus contains the nucleus 
of the levator palpebral superioris. Distal to the lateral nucleus is the 
group of cells innervating the inferior rectus and bordering on the 
nucleus of the trochlear nerve. It gives origin only to crossed fibres. 



u Marina, Neurologisches Centralblatt, September 1, 1904, p. 797. 
12 Bernheimer, Neurologisches Centralblatt, 15, 11, 1899. 



14 THE EYE AND NERVOUS SYSTEM 



Chief Nucleus 



Levator palpebrse 
Rectus superior 
Eectus interims 
Rectus inferior 
Obliquus superior „ 



> Median Line 



Most writers agree that the Darkschewitch nucleus is not a part of 
the oculomotor nucleus. No scheme of the divisions of the oculomotor 
nucleus can as yet be accepted as final. 

I cannot believe that the centre for the superior rectus is so far 
forward as is usually represented. In two cases of tumor under my ob- 
servation the upward movement of the eyeballs was much more affected 
than the downward movement. A necropsy in each case revealed a 
tumor of the pons. 

Some investigators doubt the existence of a pupillary reflex centre 
in the oculomotor nucleus, and they point out that no explanation for 
reflex pupillary rigidity has been found in the condition of the anterior 
colliculi of the corpora quadrigemina or oculomotor nucleus. Patho- 
logical alteration of the entire corpora quadrigemina has been observed 
with intact pupils, and in other cases this region has been found to 
be normal, even the Edinger-Westphal nucleus, when there had been 
reflex rigidity of the pupils or internal ophthalmoplegia of years' dura- 
tion. Eeichardt thinks there is very little evidence that the cause of 
the reflex rigidity of the pupil is to be found in the region of the oculo- 
motor nucleus, or even in the small cell median group of the oculomotor 
nucleus; indeed, contrary to such an opinion are the cases of disease 
or congenital anomaly of the region of the oculomotor nucleus without 
reflex pupillary rigidity, and the long duration of reflex pupillary 
rigidity in some cases as the only ocular sign of disease and without 
any other manifestation of disturbance of the oculomotor nucleus. 
Reichardt thinks there is more reason to believe that a lesion causing 
reflex rigidity of the pupil is to be found in the spinal cord, and this 
would explain the frequent occurrence of the sign in disease of the 
spinal cord. 

Nine cases studied clinically and pathologically by Wolff seem to 
show that degeneration of the posterior columns in the upper cervical 
region is in close relation to the rigidity of the pupils. 13 Bach', alone 
and in association with Meyer, has found from experiments on cats that 
complete even repeated division of the cervical part of the spinal cord 
several millimetres below the fourth ventricle causes no change of the 

13 Wolff, Arch, fiir Psychiatrie, vol. xxxii., No. 1, p. 57. 



%■ 




Fig. 5. — The cerebral peduncles showing the oculomotor nerves at their origin below the aqueduct 
of Sylvius, and their course as separate bundles of fibres to the point of exit at the inner side of the 
crusta. Photograph of a section from a new-born child. 





> 




Fig. 6.— The trochlear nerves decussating above the aqueduct of Sylvius. Photograph of a section 

from a new-born child. 



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Fig. 9.— Photograph of a section from the medulla oblongata of a new-horn child, showing the cochlear 
branch of the acoustic nerve at each side deeply stained in black. 



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Fig. 10. — Photograph of a section from the medulla oblongata of a new-born child, showing the vestibu- 
lar branch of the acoustic nerve at each side deeply stained in black. 




'AHrcd7vhg?n? r UAil(fn n 5^ 



Fig. 11.— Photograph of a section from the medulla oblongata of a new-born child, showing on the 
right of the picture the fibres of the glossopharyngeus or vagus entering through the spinal root of the 
trigeminal nerve. 




/4/&Gd.7?e&H*faA//*n , 



Fig. 12.— Photograph of a section from the medulla oblongata of a new-born child, showing the 
hypoglossus nerve on each side passing from its nucleus below the floor of the fourth ventricle and 
making its exit on the inner side of the inferior olive. 



THE ENCEPHALIC NERVES 15 

pupillary reaction. At the moment the division occurs the pupils be- 
come wider, but a few seconds later the light reflex is restored. 

Bilateral division of the medulla oblongata at the spinal end of the 
fourth ventricle, at a definite place, causes rigidity of both pupils to 
light. 

Division of the right half of the medulla oblongata at the spinal 
end of the fourth ventricle causes rigidity of the left pupil to light, 
while the reaction of the right pupil is preserved. 

Mere exposure of the medulla oblongata is sufficient to diminish or 
even abolish the light reflex of the pupils for a quarter of an hour to 
one hour, and when this has occurred the rigidity has promptly disap- 
peared when a cut has been made in the middle of the fourth ventricle 
or on each side in the upper part of the medulla oblongata. 

These experiments seem to show that a very limited area near the 
median line and near the respiratory centre, at the spinal end of the 
fourth ventricle, is of great importance for the light reflex of the 
pupil. This seems to be an inhibitory centre for the pupil. Cerebral 
to this area is a subordinate reflex centre of the pupil. There are, 
therefore, probably two reflex arcs for the pupil ; one passing through 
the corpora quadrigemina and one through the spinal end of the fourth 

ventricle. 

TROCHLEAR NERVE. 

The trochlear, or fourth, nerve arises in a distinct group of cells 
situated within a depression of the posterior longitudinal bundle and 
directly posterior to the oculomotor nucleus. It is separated from the 
latter group of cells. The nerve fibres on leaving the trochlear nucleus 
pass around the aqueduct of Sylvius on the same side, and somewhat- 
spinalwards, decussate above the aqueduct in the anterior medullary 
velum, and leave the brain stem as a delicate bundle directly behind 
the posterior colliculus of the corpora quadrigemina of the opposite 
side. The nerve then descends along the outer border of the cerebral 
peduncle. 

TRIGEMINAL NERVE. 

The trigeminal, or fifth, nerve is chiefly sensory, although it has also 
an important motor function in the innervation of the muscles of masti- 
cation. The motor and sensory nuclei are situated side by side, the 
motor to the inner side, in the lateral part of the tegmentum of the 
pons about midway between the cerebral and spinal ends of the pons. 
The sensory and motor bundles of this nerve are closely associated 
within the pons, so that separate strands of fibres, such as are seen in 
the intramedullary portion of the oculomotor and abdncens nerves, are 



16 THE EYE AND KEKVOUS SYSTEM 

not found in the course of the trigeminal nerve. The motor portion. of 
the nerve receives a small bundle of fibres from a group of large cells 
situated laterally to the aqueduct of Sylvius and on the same side. 

The sensory fibres arise in the Gasserian ganglion and after enter- 
ing the pons terminate only partially in the sensory nucleus, the larger 
number of them bending downward at the level of the sensory nucleus 
to form the descending or spinal root, which may be traced so far as 
the second cervical segment of the spinal cord. Within the spinal cord 
it occupies a position behind the posterior horn. The long course of 
the descending spinal root explains the implication of the trigeminal 
nerve in certain cases of lesion not extending above the medulla ob- 
longata, as in syringomyelia. 

The origin of the fibres of the spinal root in the Gasserian ganglion 
is shown by the intense degeneration that occurs in these fibres when 
the sensory root of the nerve is cut between the pons and the Gasserian 
ganglion. 

The sensory fibres on entering the pons pass dorsally and medially 
to the sensory nucleus. The motor root exterior to the pons remains 
distinct until it becomes united with the third division of the trigeminal 
nerve at the peripheral end of the Gasserian ganglion. 

ABDUCENS NERVE. 

The abducens, or sixth, nerve has its nucleus beneath the floor of 
the fourth ventricle in the knee made by the fibres of the facial nerve 
as they pass upward and then outward. The nucleus is well defined, 
almost spherical, and contains numerous cells. It is not probable that 
any of the axis cylinder processes of these cells are associated with the 
fibres of the facial jaerve. 

The fibres of the abducent nerve on leaving the dorsal and inner 
sides of the nucleus pass ventrally and make their exit at the junction 
of the pons with the medulla oblongata and near the median line. The 
nucleus is situated a little more cerebralward than is the point of exit 
of the nerve, consequently the fibres pass a little spinalward in their 
exit from the brain stem. The fibres are not united into one bundle in 
their course through the tegmentum of the pons and transversely 
through the pyramidal tract. They do not decussate on leaving the 
nucleus, although, according to some anatomists, they may receive a 
small addition from the nucleus of the other side. 

The close association of the abducent nucleus with the fibres of the 
facial nerve explains the occurrence of paralysis in the distribution of 
these two nerves when a lesion is situated in the lower part of the 



PLATE B. 




".'--/ 






a, olfactory nerve ; b, optic nerve ; c, optic tract ; d, oculomotor nerve ; e, trochlear nerve ; /, sensory 
portion of trigeminal nerve ; g, motor portion of trigeminal nerve ; h, abducens nerve ; i, facial and acous- 
tic nerves ; j, glossopharyngeal, vagus and accessorius nerves; k, hypoglossus nerve ; I, first cervical root; 
to, second cervical root. 



THE ENCEPHALIC KEBVES 17 

tegmentum of the pons. The nearness of the two abducent nerves to 
one another at their point of exit from the pons explains the occurrence 
of paralysis of both external rectus muscles in basal meningitis. 

The abducent nucleus receives fibres from the superior olive, which 
is believed to be in connection with the acoustic nerve, and in this way 
a functional relation may be established between the two nerves. It is 
also in connection with that portion of the oculomotor nucleus which 
controls the internal rectus muscle of the opposite side. This connec- 
tion is probably by means of the posterior longitudinal bundle. 

At the point of exit, at the junction of the pons and medulla ob- 
longata, the fibres of the abducent nerve become united into one bundle. 
From here to the exit from the skull through the sphenoidal fissure the 
course of the nerve is long, and consequently paralysis of the external 
rectus muscle is not uncommon from intracranial lesions. 

FACIAL NERVE. 

The facial, or seventh, nerve arises from a large group of- cells situ- 
ated in the upper part of the medulla oblongata at about where this 
structure passes into the pons. The nucleus is situated postero-laterally 
to the upper end of the lower olive. The fibres arising in the cells of 
this nucleus pass posteriorly and medially ununited toward the raphe 
to just below the floor of the fourth ventricle. Here the fibres ascend 
and bending again so as to form a knee, in which is situated the nucleus 
of the sixth nerve, pass ventro-laterally and on the inner side of the 
spinal root of the fifth nerve to their exit point at the junction of the 
medulla oblongata and pons. The fibres of this nerve at their exit 
from the pons lie on the inner side of the acoustic nerve. The loop 
made by the facial nerve within the lower part of the tegmentum of 
the pons is one of the most remarkable formations within the central 
nervous system, and seems to serve no definite purpose. The glosso- 
pharyngeus and vagus nerves have a similar course. From the knee 
onward the fibres of the facial nerve are united in a distinct bundle. 

It has not been possible to distinguish a nucleus for the upper 
distribution of the facial nerve separate from that for the lower dis- 
tribution ; indeed, it seems probable that the entire facial nerve has one 
common nucleus in the lower part of the pons, although different 
branches of the nerve may be represented in different portions of the 
nucleus. 

ACOUSTIC NERVE. 

The acoustic, or eighth, nerve consists of two distinct parts, the coch- 
lear nerve concerned with hearing and the vestibular nerve concerned 

2 



18 THE EYE AKD KEKVOUS SYSTEM 

with coordination. The latter enters the medulla oblongata on the 
inner side of the restiform body; the former passes on the outer side 
of this structure. The cochlear nerve extends further spinalward than 
does the vestibular branch, but the latter extends further cerebralward 
than does the cochlear branch. The fibres of the cochlear nerve termi- 
nate about the cells of the ventral nucleus and the acoustic tubercle. 
The ventral nucleus lies on each side of and within the substance of 
the cochlear nerve at its entrance into the medulla oblongata. The 
acoustic tubercle lies a little dorsally to the ventral nucleus, and in man 
is not sharply separated from it. The trapezoid body and the acoustic 
striae form a part of the system connecting the cochlear nerve with 
structures situated higher in the brain. The former passes ventrally 
to the restiform body towards the raphe ; the latter pass around the 
restiform body. The trapezoid body is much less important in man 
than in some of the lower animals. It is in connection with the. trape- 
zoid nucleus and superior olive, and by another system of fibres, the 
lateral fillet, is brought into connection with the posterior colliculus of 
the corpora quadrigemina. 

The vestibular nerve enters the medulla oblongata between the 
restiform body and the descending spinal root of the trigeminal nerve. 
The fibres of this nerve terminate about the cells of the posterior nu- 
cleus below the floor of the fourth ventricle, and in the nuclei of Deiters 
and von Bechterew, below and lateral to the floor of the fourth ventricle. 
The vestibular nerve possesses also a descending root, between the 
fibres of which are numerous nerve-cells. 

GLOSSOPHARYNGEAL AND VAGUS NERVES. 

The glossopharyngeus, the vagus, and the cerebral portion of the 
accessorius cannot be sharply separated at their exit from the medulla 
oblongata. The fibres of these nerves are found directly dorsal to the 
lower olive and in the continuation downward of this line of exit. The 
uppermost fibres belong to the glossopharyngeus; the middle to the 
vagus, and the lowest to the accessorius. The fibres are not united into 
three nerve bundles at their point of exit from the medulla oblongata. 

The sensory glossopharyngeus and sensory vagus nerve-fibres enter 
the medulla oblongata laterally, passing through the spinal root of the 
trigeminal nerve. Some of these terminate in the dorsal nucleus below 
the floor of the fourth ventricle and lateral to the hypoglossus nucleus. 
Others bend downward near the lateral part of the fourth ventricle to 
form the descending root. This root is accompanied by a small amount 
of gray matter, and about the nerve-cells of this many of the fibres of 



THE ENCEPHALIC XERVES 19 

the descending root terminate. In the opinion of some anatomists, the 
descending root contains chiefly glossopharyngeus fibres and few vagns 
fibres. 

The posterior nucleus is regarded by some anatomists as motor in 
function and not sensory. The ventral nucleus, or nucleus ambiguus, 
is in all probability motor. This nucleus is not sharply defined. It 
lies dorso-laterally to the lower olive. In some transverse sections only 
a few large cells belonging to this nucleus may be seen; in other sec- 
tions no cells at all are found. The nerve-fibres arising in this nucleus 
pass separately dorsally and medially, much in the same way as do the 
fibres of the facial nerve, but before reaching the fourth ventricle bend 
to form a knee, and join the sensory fibres passing obliquely through 
the medulla oblongata. 

ACCESSORIUS NERVE. 

The accessorius, or eleventh, nerve has been described so far as its 
cerebral portion is concerned in connection with the glossopharyngeus 
and the vagus. The spinal portion arises in the lateral horn of the 
spinal cord, and the nerve-fibres passing from the cells in this situa- 
tion reach the periphery of the cord through the lateral column. The 
nerve-fibres can be observed leaving the cord as separate strands as low 
as the fifth or sixth cervical segment. 

The upper fibres, or cerebral portion, of the accessorius probably 
belong to the vagus nerve. 

HYPOGLOSSAL NERVE. 

The hypoglossus, or twelfth, nerve has its nucleus just below the floor 
of the fourth ventricle close to the raphe. It is a well defined group of 
large nerve-cells, resembling closely those of the anterior horns of the 
spinal cord. The nerve-fibres arising in these cells pass a little 
obliquely forward in straight lines until they reach the lower olive, but 
they are more or less diverted from their course in passing through this 
structure. They leave the medulla oblongata lateral to the pyramid, 
although some of the nerve-fibres pass directly through the pyramid. 

The nucleus of the hypoglossus is one of the easiest to find of all the 
motor nuclei in the medulla oblongata and pons, and is about 18 milli- 
metres in length, extending upward about as far as the stria? medullares. 



CHAPTEE II. 

SIGHT : THE STRUCTURE AND OPTICAL PROPERTIES 

OF THE EYE. 

By WILLIAM NORWOOD SOUTER, M.D. 

Sight, or vision, is that sense by which we appreciate the form and 
color of objects through the action of the physical energy, light, on the 
retina. In other words, sight is the consciousness which results from 
stimulation of the retina by light. The visual apparatus, therefore, 
consists of two distinct parts : ( 1 ) the eye, which is comparable to a 
photograph camera, the retina (which receives and transforms the light- 
energy into a nerve-impulse) corresponding to the sensitized plate of 
the camera; and (2) the conducting and interpreting mechanism, by 
means of which the nerve-impulse is conducted to the visual areas of the 
brain and thence to the centres of consciousness where the impulse is 
manifested as sight. 

The manner in which the light-stimulus is transformed into a nerve- 
impulse is but little understood; it is only known that in this trans- 
formation a certain chemical change (destruction of the retinal purple) 
takes place in the retina. The second transformation, that of the nerve- 
impulse into the consciousness of vision, is also beyond our compre- 
hension. So much as is known of the mental process of vision will be 
considered in a subsequent chapter ; it is the province of the writer of 
the present chapter to consider only the structure of the eye, and its 
adaptation and imperfections as an optical mechanism. (See chapter in.) 

THE STRUCTURE OF THE EYE. 

The eyeball is spheroidal in shape. Its normal diameter varies be- 
tween 23 millimetres and 25 millimetres. The antero-posterior diam- 
eter is slightly greater than the transverse diameter, while the vertical is 
the least of the three dimensions. The difference between the greatest 
and the least diameter is about 1 millimetre. 

As externally viewed the eye is divisible into two distinct portions : 
an anterior, transparent portion, or cornea, and a posterior, larger, 
opaque portion, the sclera. The curvature of the cornea is greater than 
that of the sclera, so that at the junction of these two structures there is 
formed a groove or sulcus — the sulcus corneas. On antero-posterior 
20 



STRUCTURE AND OPTICAL PROPERTIES 21 

meridional section of the eye the corneal portion comprises about one- 
sixth and the scleral portion the remaining five-sixths of the circum- 
ference. 

The eyeball is protected posteriorly by the bony walls of the orbit, 
but antero-exteriorly there is no other protection than that afforded by 
the lids. The latter serve, when partially or completely closed, to pro- 
tect the eye from excessive light and to some extent from injury by 
foreign bodies. The inner surface of the lids is covered with mucous 
membrane, the conjunctiva, which is reflected at the upper and lower 
fornices (the upper and lower culs-de-sac) upon the eyeball, and the 
epithelium of this membrane is continued over the anterior surface 
of the cornea. The conjunctiva thus forms a sac, open anteriorly at the 
lid-margins. 

There are three concentric coats or tunics of the eye : the external, 
the middle, and the inner coat. 

The External Coat. — The external coat consists of the transparent 
cornea anteriorly and the opaque sclera posteriorly. The structure of 
these two membranes is essentially the same, both being of a fibrous 
nature. The transparency of the cornea is the result of close union and 
regular arrangement of the fibres, and of homogeneity of refractive 
index of the component corneal tissues. 

The Cornea is covered anteriorly by epithelium, which is continuous 
with the epithelium of the conjunctiva. Immediately behind the epi- 
thelium is the anterior limiting membrane (Bowman's membrane), 
which is a dense, structureless membrane. Next to this is the corneal 
stroma, which consists of regularly arranged lamellae of fibrous tissue 
having lymph spaces or lacunce connected by small canals or canaliculi. 
In each lacuna there is situated a cell whose processes extend along the 
canaliculi so as to communicate with adjacent cells. These are the fixed 
cells of the cornea, in contradistinction to the leucocytes or movable 
cells which are carried along the lymph channels. The fourth layer 
of the cornea is a thin, homogeneous, very resistant membrane, known 
as Descemefs membrane. It is continuous with the ligamentum pecti- 
nation (p. 23), which, together with Descemet's membrane, is embryo- 
logically a part of the middle coat of the eye. The fifth and innermost 
layer is the endothelium, or layer of polygonal cells, which is continuous 
with the endothelium of the iris, and which, like the preceding layer, is 
embryologically a part of the middle coat. There are no blood-vessels in 
the healthy cornea, nourishment being supplied through lymph channels. 

The corneal nerves are derived from the ciliary plexus. They pene- 
trate the sclera on the outer side of Schlemm's canal and form a net- 



22 THE EYE AND NERVOUS SYSTEM 

work in the vicinity of the corneal margin. Erom this plexus branches 
are given off which supply the several layers of the cornea. 

The radius of curvature of the anterior surface of the cornea is about 
8 millimetres, 7.8 millimetres being the commonly accepted average for 
the normal eye. The radius of curvature of the posterior surface is 
about 6 millimetres. The thickness of the cornea at its centre is about 1 

millimetre. 

The sclera is, as previously stated, composed of dense fibrous tissue 
irregularly arranged. It is of a glistening whitish color, which indi- 
cates its scant blood supply. The sclera is pierced by a number of 
openings for blood-vessels and nerves. The anterior ciliary arteries, 
veins, and nerves enter the eyeball near the margin of the cornea. In 
the equatorial region there are four or five openings for the passage of 
the vense vorticosse. Posteriorly there are a number of small openings 
for the posterior ciliary arteries and veins, and a large opening for the 
optic nerve. This opening is about 1.5 millimetres in diameter, and is 
situated slightly to the nasal side of the posterior pole of the eye ; it is 
traversed by connective-tissue fibres, continuous with the inner layers 
of the sclera, which are known as the lamina cribrosa. The outer layers 
of the sclera are continuous with the sheath of the optic nerve. 

The greatest thickness of the sclera is about 1 millimetre, near 
the posterior pole. Erom this region it becomes somewhat thinner 
toward the equator, and is again thickened anteriorly where the tendons 
of the extraocular muscles blend with the sclera. 

Anteriorly, near the sclero-corneal junction and concentric with it 
is found the circular sinus, known as Schlemms canal. The nature of 
this was for a long time in doubt, but it is now regarded as a venous 
channel, into which drains also the aqueous humor through the ligamen- 
tum pectinatum. 

The sclera is covered externally by a thin layer of loose connective 
tissue, the episclera, which is more freely supplied with blood-vessels 
than the sclera proper. 

The anterior portion of the sclera is covered by conjunctiva which 
is reflected from the lids at the upper and lower culs-de-sac; the poste- 
rior portion is embedded in Tenon's capsule, from which it is separated 
by a lymph space, thus allowing the eye to be freely movable in the 
capsule. 

The Uvea. — The middle coat or uvea is so named from its resem- 
blance (when stripped of the sclera) to a grape. 

The iris or anterior portion of the uvea is a diaphragm having in it 
a circular opening (the pupil) for the passage of light to the retina. 



STRUCTURE AKD OPTICAL PROPERTIES 23 

Erom the free pupillary border the iris extends peripherally to the 
ciliary body, to which it is attached slightly behind the sclero-corneal 
junction. The angle between the sclero-cornea and the root of the iris 
is bridged over by a loose, spongy tissue, called the ligamentum pectina- 
tum. The patulency of this structure is of great importance in the 
regulation of intraocular tension, since through its meshes the aqueous 
humor is drained off into Schlemm's canal, of which the ligamentum 
pectinatum forms the inner wall. 

The iris is composed of an endothelial layer in front (continuous 
with the endothelium of the cornea), a pigment layer behind (continu- 
ous with the pigment layer of the retina), and, between these two, the 
stroma. The latter is a network of blood-vessels and fibrous tissue, in 
which are embedded the two muscles of the iris, the sphincter and the 
dilator. The former is a circular band of muscular tissue about 1 
millimetre in width, which surrounds the pupil and causes contraction 
of this opening. The dilator muscle consists of longitudinal or radial 
fibres which assist in dilating the pupil. These two muscles regulate 
the size of the pupil, which contracts in brilliant illumination to cut off 
the excess of light, and expands in feeble illumination to allow sufficient 
light to enter the eye. 

The bluish color of the iris in blondes is due to the pigment con- 
tained in the posterior or retinal pigment layer ; in dark eyes there is 
additional pigment in the stroma. 

The ciliary body, the middle portion of the uvea, is ring-shaped, tri- 
angular on transverse section, with its broadest side or base in apposi- 
tion with the inner surface of the sclera (Fig. 1). This body extends 
from the sclero-corneal junction backward for a distance of about 2 
millimetres, being merged in the chorioid at the ora serrata, or anterior 
extremity of the retina. The ciliary body is composed of the ciliary 
processes and the ciliary muscle. The ciliary processes are connective- 
tissue elements in which are embedded a large number of blood-vessels 
and pigment-cells. There are from fifty to seventy of these processes 
longitudinally arranged with their larger ends projecting anteriorly. 
The ciliary muscle lies beneath the processes and consists of two parts. 
The larger part is formed of meridional fibres (Briiel'e's muscle) which 
are attached anteriorly with firm union to the sclero-corneal junction 
and neighboring sclera. The fibres of this part pass backward and are 
inserted into the anterior portion of the chorioid. This is the most 
external part of the muscle. To its inner side and adjoining the pro- 
cesses is the second or transverse part, the annular muscle of Muller, 
which consists of a band of muscular fibres surrounding the periphery 



24 



THE EYE AND NERVOUS SYSTEM 



of the iris. This part constitutes about one-tenth of the entire muscle 
in the normal or emmetropic eye. In hyperopia the annular muscle is 
larger and in myopia it is smaller than in emmetropia. The ciliary 
muscle is an essential factor in the accomplishment of accommodation. 
By contraction of this muscle the crystalline lens becomes more convex, 
so as to enable near objects to be seen clearly. 

The chorioid is that part of the uvea which is posterior to the ciliary 
body. It is dark brown in color, thus differing from the ciliary body, 
which is black; its inner surface is smooth, while the outer surface 
when torn from the sclera presents a shaggy appearance. In this respect 
and also in the abundance of blood-vessels the chorioid resembles the 



Canal of Schlemm 
^^^§^ ^ Spaces of Fontana 
Ant. Ciliary Veins 

Conjunctn 

Sclera 




Fig. 1.— Diagram of anterior segment of eye, drawn to accurate scale. (Flemming.) 



embryonic chorion, from which the name is derived. The thickness of 
the chorioid varies from .08 millimetre near the optic nerve to .01 
millimetre at the ora serrata. 

In structure the chorioid consists of a network of blood-vessels, 
intervascular connective tissue, and pigment. It is usually described 
as consisting of five layers, which, beginning with the external layer, 
are: (1) The supra-chorioid, (2) the layer of large vessels, (3) the 
layer of medium vessels, (4) the layer of capillaries, and (5) the 
lamina vitrea. 

The supra-chorioid is composed of non-vascular but richly pigmented 
lamellae, which lie between the sclera and the chorioid proper. When 
stripped from the sclera these lamellae are torn in shreds, from which 
the chorioid acquires its shaggy appearance. 



STRUCTURE AXD OPTICAL PROPERTIES 25 

The layer of large vessels, consisting chiefly of veins, is placed in a 
close network, with many anastomoses. The intervascular spaces are 
highly pigmented. 

The layer of medium-sized vessels is thin and only slightly pig- 
mented. • 

The capillary layer consists of large capillaries, closely arranged. 
This layer contains no pigment. 

The lamina vitrea is a thin homogeneous membrane which coats the 
inner surface of the capillary layer. 

The blood supply of the uvea is derived from the anterior and the 
posterior ciliary arteries. The anterior ciliary arteries, four in num- 
ber, arise from the four arteries supplying the recti muscles, pierce the 
sclera near the cornea, and terminate in the greater arterial circle of 
the iris. Erom this circle radial branches are given off to supply the 
iris, and these, by reunion, form the lesser arterial circle near the 
pupillary border of the iris. The posterior ciliary arteries consist of the 
long and the short ciliary arteries. Of the former there are two, which 
arise from the ophthalmic artery, pierce the sclera near the optic nerve, 
and continue forward, one on each side of the eyeball, between the 
sclera and chorioid as far as the ciliary muscle. Each artery then divides 
into two branches, which form, with the aid of the anterior ciliary 
arteries already mentioned, the greater arterial circle of the iris. It 
thus appears that the anterior and the long posterior ciliary arteries sup- 
ply chiefly the iris and ciliary body. The short posterior ciliary arteries, 
from twelve to fifteen in number, arise from the ophthalmic or some 
of its branches, and surround the optic nerve as they pass to the posterior 
part of the eyeball, where they pierce the sclera and supply the chorioid 
and ciliary processes. 

The greater part of the venous blood from the uvea leaves the eye 
through the venae vorticosa?, of which there are from four to six pene- 
trating the sclera in the equatorial region of the eyeball. A much 
smaller proportion of the venous blood, chiefly that from the ciliary 
muscle, leaves the eye through the anterior ciliary veins, which pierce 
the sclera near the cornea and pass backward under the conjunctiva. 
These veins anastomose with the conjunctival veins and also with 
Schlemm's canal. 

The nerves of the uvea are the long and the short ciliary nerves. 
The former, two or three in number, are derived from the nasal branch 
of the fifth nerve. The short ciliary nerves, from ten to twelve in 
number, are derived from the ciliary ganglion ; they contain motor 
fibres from the third nerve, sensory fibres from the fifth nerve, and 
fibres from the sympathetic system. 



26 



THE EYE AKD 1STEKV0US SYSTEM 



The Retina. — The retina is the third or innermost eye-tunic. It is 
formed by the terminal expansion of the optic nerve, and, with the 
exception of its outer or pigment layer, is transparent during life, hav- 
ing only a slight purplish tinge (the retinal purple), which fades on 
exposure to light. 

The retina extends forward as far as the ora serrata, beyond which 
it exists only in rudimentary form. In the latter condition it extends 
to the border of the pupil, furnishing the posterior pigment layer of 
the ciliary body and iris (pars ciliaris retince and pars iridica retinas) , 




Fig. 2.— Section of human retina, showing the usual appearance of the component layers in ordinary- 
preparations.— a, pigment layer; b, rods and cones; c, external limiting membrane ; d, outer nuclear 
layer; e, outer plexiform layer; /, inner nuclear layer ; g, inner plexiform layer; h, layer of ganglion- 
cells ; i, fibre layer ; Jc, internal limiting membrane ; I, base of Miller's fibre ; v, blood-vessel. Magnified 
475 diameters. 



which is continuous with the pigment layer of the retina proper (pars 
optica). This pigment layer is embryologically a part of the retina, 
but it adheres to the chorioid when the retina is lifted from the former 
membrane. 

The fibres of the optic nerve, which within the eye consist only of 
axis-cylinders (except in the anomalous condition known as opaque 



STRUCTURE AKD OPTICAL PROPERTIES 



27 



nerve-fibres), radiate from the optic dish, or entrance of the optic nerve, 
and form the inner layer of the retina. The optic disk is situated 
slightly to the nasal side of the posterior pole of the eye. At the poste- 
rior polar region is situated the macula lutea or yellow spot. This is 
described as an annular or oval area from 1 millimetre to 2 millimetres 
in diameter, at the centre of which lies a minute depression, the fovea 
centralis. 



Adjacent to the chorioid. 




Adjacent to the vitreous. 

Fig. 3.— Scheme of the structure of the human retina. 

A. Horizontal section, haematoxylin stain. I. Pigment epithelial layer. II. Layer of rods and 
cones: a. external; b. internal elements. III. External limiting membrane. IV. External molecu- 
lar layer : c, fibre layer. V. External granular layer. VI. Internal molecular layer : d, spongio- 
blasts ; e, supporting fibres of Miiller ; /, nuclei of the same. VII. Internal granular layer. VIII. 
Layer of ganglion cells. IX. Nerve-fibre layer. X. Internal limiting membrane. 

B. Demonstration after the method of Golgi. I. Pigment epithelial layer. II. Layer of rods and 
cones. III. Molecular and visual cells. IV. External plexiform layer. V. Layer of horizontal cells. 
VI. Layer of bipolar cells. VII. Layer of amacrine cells. VIII. Internal plexiform layer (fibre layers). 
IX. Layer of ganglion cells. X. Nerve-fibre layer : 1, diffuse amacrine cells; 2, diffuse ganglion cells ; 
3, centrifugal nerve fibres ; 4. association-amacrine cells ; 5, neuroglia cells ; 6, supporting fibres of 
Miiller. 



In structure the retina is composed of an outer and an inner lamina. 
The outer lamina constitutes the retinal pigment layer. The inner 
lamina constitutes the rest of the fully formed retina (Fig. 2). The 
inner lamina is subdivided into two layers, the neuro-epithelial and 
the cerebral (Eig. 3). The neuro-epithelial layer consists of the 



28 



THE EYE AND NERVOUS SYSTEM 



retinal rods and cones, which constitute the light-receiving and trans- 
forming mechanism. The cerebral layer, which is the impulse-trans- 
mitting part of the retina, contains a layer of bipolar cells and a layer 
of ganglion-cells and nerve-fibres. The bipolar cells connect by their 
processes the rods and cones on the one hand with the ganglion-cells of 
the nerve-fibres on the other, and thus form the connecting link between 
the peripheral recipient elements and the cerebral conducting nerve- 
fibres. The bipolar cells are not united to the rods and cones or to the 
ganglion-cells, but their process-terminals are in close apposition with 
the terminals of the elements which they connect. 

In addition to the bipolar cells just described there are also found 
similar cells, except that their processes extend horizontally. From 



External surface 




Fro. 4.-Diagrammatic section of the human fovea. Magnified 375 diameters. ( Golding-Bird and 
Schafer.)-2, ganglion layer ; 4, inner nuclear layer ; 6, outer nuclear layer, the cone-fibres forming the 
so-called external fibrous layer of Henle ; 7, cones ; v, section of a blood-vessel ; M, membrana limitans 
interna ; og, ig, outer and inner granules (cone-nuclei and bipolars) at the centre. 



their apparent nerve connections these cells are called association cells, 
though their function is not definitely known. Still other cells are 
found which apparently have no nerve connections, and whose function 
is likewise not known. They are called spongioblasts (Miiller) or ama- 
crine cells (Cajal). • 

The supporting neuroglia or sustentacular tissue of Miiller consists 
of transverse fibres and an outer and an inner membrane, the external 



STRUCTURE AND OPTICAL PROPERTIES 29 

and the internal limiting membrane, respectively. These two mem- 
branes, together with the retinal pigment layer and the various sub- 
divisions of the neuro-epithelial and cerebral layers, make up the ten 
layers into which the retina is usually divided. 

The macula lutea, that part of the retina which is concerned in dis- 
tinct vision, requires special description. In this region the rod-ele- 
ments are replaced by cones, so that well within the border of the macula 
there are no rods, while the number of cones is very great. The layer of 
ganglion-cells is also much thickened in this area, except at the fovea, 
the region of greatest visual acuteness, where the ganglion-cell layer 
becomes very thin ; at the foveal centre these cells are not found, the 
connection between the cones and the nerve-fibres being made by lat- 
erally extending processes of the bipolar cells. Transversely the pro- 
cesses of these cells are much shorter at the fovea than elsewhere. This 
fact, together with the diminution of the ganglion-cells, makes the 
cone-elements the most prominent feature at the fovea. The cone- 
fibres and nuclei, constituting the outer nuclear layer (iv, Fig. 3, A) 
are relatively conspicuous; they form the external fibrous layer of 
Henle. The fibres are situated obliquely at the sides of the fovea, but 
perpendicularly at its centre (Fig. 4). In the latter situation the 
fibres are much shorter than at the sides, thus adding another factor in 
the causation of the depression at the fovea ; the principal other factors 
are the thinning of the nerve-fibre layer (ganglion-cells) and the sessile 
character of the bipolar cells. 

Blood Supply of the Retina. — The outer layers of the retina receive 
their blood supply from the capillaries of the adjacent chorioid. The 
inner layers are supplied by the central artery of the retina. This 
artery is derived from the ophthalmic artery; it penetrates the optic 
nerve about 5 millimetres behind the eyeball and emerges at the optic 
disk, slightly on the nasal side of its centre. It is divided at its point 
of emergence or slightly behind the disk into two branches, an upper 
and a lower branch. These branches traverse the disk giving off small 
branches, and again divide just within or without the border of the 
disk into temporal and nasal branches. The further subdivisions are 
illustrated in Fig. 5. The branches of the retinal artery are terminal, 
that is, there are no anastomoses, and consequently embolism of a 
branch is followed by loss of function. 

The efferent veins, slightly larger than the arteries, follow the same 
general course as the arteries. Although there are no large vessels at 
the macula, this region is abundantly supplied with capillaries, except 
at the fovea, where there are no blood-vessels. 



30 



THE EYE AND NEKVOUS SYSTEM 



Refractive and Distensive Contents of the Eye. — Within the inner 
tunic of the eye just described are included the aqueous humor, the 
crystalline lens, and the vitreous body. These substances serve the dou- 
ble purpose of keeping the otherwise collapsible eyeball distended and 
at the same time of acting as refractive media. 

The aqueous humor is a transparent, slightly alkaline fluid, com- 
posed chiefly of water and sodium chloride ; it is secreted by the ciliary 




Fig. 5.— Diagram of the blood-vessels of the human retina. (Leber, after Jaeger. )—ans, vns, superior 
nasal artery and vein; ats, vts, superior temporal artery and vein; ani, vni, inferior nasal vessels; 
ati, vti, inferior temporal vessels ; ame, vme, median vessels ; am, vm, macular vessels. 



body, passes forward through the pupil (Fig. 1), and is carried of! 
through the interspaces of the ligamentum pectinatum into Schlemm's 
canal. 

The crystalline lens is formed of delicate fibres closely united. In 
childhood the lens substance is gelatinous. In early adult life the 
central part or nucleus is solid, while the outer or cortical part is soft. 
In old age the entire lens is solid. The fibres are arranged in regular 
layers, and the lens is divisible into three principal segments. With 
the diminution of transparency which takes place in old age, the lines 
of union of these segments become visible when the lens is examined 
with the aid of oblique illumination. 

The lens is enclosed in a delicate capsule, to which is attached, 
anteriorly and posteriorly, near the periphery or equator of the lens, 



STEUCTUKE AXD OPTICAL PROPERTIES 31 

the suspensory ligament (zonule of Zinn). By this ligament, the outer 
border of which is attached to the ciliary body, the lens is held in posi- 
tion (Fig. 1). 

In shape the lens is biconvex, its anterior surface being less curved 
than its posterior surface. The radius of curvature of the anterior 
surface is about 10 millimetres, while that of the posterior surface is 
about 6 millimetres. In childhood, and to a less degree at a later age, 
prior to complete solidification, the curvature of the anterior surface of 
the lens is capable' of increase by contraction of the ciliary muscle and 
consequent relaxation of the suspensory ligament. In this way the eye 
is adapted for different distances as is required in near vision. 

The lens is a bloodless body after birth, and its metabolism is ex- 
tremely slow, especially in its inner or nuclear portion. It is probable 
that as much nutritive fluid as is required is supplied from the adjacent 
ciliary body. 

The vitreous body, which occupies the larger and posterior part of 
the eye, consists of a transparent, semi-fluid, colloid substance, together 
with trabecule of supporting tissue. The whole is enclosed in a cap- 
sule, the hyaloid membrane, on the anterior, concave surface of which 
the crystalline lens rests. The vitreous also contains floating cells 
(muscce volitantes) which can usually be seen entoptically when one 
looks at a uniformly bright light, as the sky. In disease these cells 
sometimes become greatly multiplied, so as seriously to interfere with 
vision. 

Like the lens, the vitreous is devoid of blood after birth, and conse- 
quently must receive its nourishment from surrounding tissues, the 
ciliary body and chorioid. There is also a central canal (which lodges 
the pre-natal hyaloid artery) running from the posterior pole of the 
lens to the optic disk and communicating with the lymph spaces of the 
optic nerve. This canal doubtless plays an important part in the metab- 
olism of the vitreous. 

THE EYE AS A REFRACTIVE APPARATUS. 

In order that we may perceive the form of an object by the visual 
sense the stimulated area on the retina must constitute an image of 
that object. This image is formed by the refracting apparatus of the 
eye. It is well known that light appears to travel in straight lines 
(rays) as long as the medium is unchanged, but when light passes from 
one medium to another of different density or refractive index the 
path of all rays which meet the separating surface obliquely undergoes 
a change of direction. This change in direction is called refraction; it 



32 



THE EYE AND NERVOUS SYSTEM 



is explained by assuming that light travels in waves and that the progress 
of these waves is less rapid in dense than in rare substances. 

By means of a properly curved surface separating two media a 
considerable portion (a pencil) of the rays proceeding from a point may 
be united in another point or focus after refraction, as is illustrated in 
Fig. 6, in which SAS' represents a section of a spherical surface sep- 



Fig. 6. 




B' s' 



arating two media, the second medium (that on the right of the figure) 
being denser than the first, while SOS' represents a section of a pencil 
of light proceeding from 0. That portion of the wave which travels 
along OA encounters the denser substance sooner than the portions 
which travel along OS and OS'. While the first portion travels the 
distance from A to H in the denser substance, the peripheral portions 
travel the greater distances BS and B'S' in the rarer medium. When 
the whole pencil has entered the denser medium SHS' represents the 
form of the wave front, and SI, 01, and S'l represent rays (perpendic- 
ular to the wave front), all meeting in the focus, I. The ray 01, which 
meets the separating surface perpendicularly and which consequently 
undergoes no refraction, is called the optic axis. 

The position of the focus / depends upon the degree of refraction, 
— that is, upon the curvature of the surface and the relative densities 

Fig. 7. 




(refractive indices) of the two media. Eor the same surface and media 
the position of the focus also varies with the point of origin of the 
incident pencil. As the point becomes more distant from the sur- 
face the focus / approaches the surface, and vice versa. The two points 
and / are called conjugate foci. When the anterior focus is so far 
distant that the wave front may be regarded as plane and the rays as 



STRUCTURE AXD OPTICAL PROPERTIES 



33 



parallel, the conjugate focus is called the posterior principal focus 
(Fig. 7). When, on the other hand, the point approaches so near 
the surface that the rays are parallel after refraction, that is, when the 
conjugate / is infinitely distant, the point from which the rays proceed 
is called the anterior principal focus (Fig. 8). When the point of 
origin is nearer than the first principal focus the rays will remain 

Fig. 8. 




divergent after refraction, but the divergence will be less than before 
refraction. In this case the rays appear to proceed from a point, /, on 
the left of (Fig. 9), but since they do not actually pass through this 
apparent point of origin, this is not an illuminated point as is the focus 
/ in Fig. 6. Hence the latter is called a real focus, while the former is 
a virtual focus. So also, if the rays have been rendered convergent by 
previous refraction, so that they are already converging toward a focus, 
the convergence of the rays will be increased by the refraction at the 
convex surface (provided the focus toward which the rays converge is 
beyond the centre of the surface), so that after this refraction the rays 
will meet in a real focus which is nearer the surface than the principal 

Fig. 9. 




focus. This condition is represented in the correction of hyperopia by 
a convex lens, as is illustrated in Fig. 14, p. 41. The path of the 
light motion may be reversed in any of the preceding diagrams ; that 
is, these diagrams may be used to illustrate the refraction which takes 
place when light passes from a dense to a rare medium at a concave 
surface. On the other hand, it is apparent that the refraction which 
takes place when light passes from a rare to a dense medium at a con- 
cave surface or from a dense to a rare medium at a conrc.r surface is 
3 



34 



THE EYE AND JSTEKVOUS SYSTEM 



opposite to that hitherto described; that is, such refraction has the 
effect of increasing the divergence or diminishing the convergence of 
rays. 

As the various rays proceeding from (Fig. 10) are concentrated 
at 7, so the rays from 2 are focused at I l9 and those from 2 are focused 
at 7 2 . Hence 7 2 7 2 is the image of O x 2 . All the rays which pass 
through the centre C meet the surface perpendicularly and consequently 
are unrefr acted. The point C is called the nodal-point and the unre- 
fracted rays are called nodal-rays, or optic axes, 01 being the principal 
axis and 1 I 1 and 2 7 2 secondary axes. 

Since O-lOoC and I^T^C are similar triangles, we know that the 
linear dimensions O x 2 and 7 1 7 2 are proportional to the distances OC 






___ . — " ' A 


Fig. 10. 

S 


— — ______ 




4 




S 



and 7(7, or each linear dimension of the image bears the same propor- 
tion to the corresponding dimension of the object as does the distance 
of the image from the nodal-point to the distance of the object from this 
point. Hence it is apparent that the nearer an object is to the refract- 
ing surface (as the eye) the larger. will be the image as formed after 
refraction. 

The cornea of the eye presents a curved (approximately spherical) 
surface, convex to incident light, and the refractive index of the cornea 
and aqueous humor is greater than that of air ; 1 hence the condition of 
refraction when light enters the cornea is similar to that represented in 
Fig. 10. The corneal refraction, however, is not sufficiently great to 
bring even parallel rays to a focus on the retina of the normal eye, and 
still less would it be able to focus rays from a near object. The de- 
ficiency is supplied by the crystalline lens, whose refractive index is 
higher than that of the aqueous and vitreous, and which is biconvex in 



1 The refractive index of the aqueous is practically identical with that of the 
vitreous, while the index of the cornea is slightly greater than that of the aqueous 
and vitreous; but for the sake of simplicity a common index (1.337) may be 
assumed for these three media. 



STRUCTURE AXD OPTICAL PROPERTIES 35 

form. By the additional convergence given rays of light in passing 
through this lens they are brought to a focus on the retina in the normal 
eye, and consequently a well-defined image of the object of vision is 
formed on the retina. 

A combination of several surfaces and media, such as is formed by 
the cornea, aqueous, lens, and vitreous, constitutes a compound optical 
system. Such a system is analogous to a simple system of one surface 
and two media, except that, instead of both conjugate focal distances 
being measured from the same point (where the surface and principal 
axis intersect), the anterior and posterior focal distances are measured 
respectively from the first and the second principal point, and instead 
of a single nodal-point there are two nodal-points, separated by the same 
interval as the principal points. In a compound system the nodal-ray 
does not form a continuous straight line, but its characteristic is that 
after the last refraction its direction is parallel to that which it had 
before entering the refracting system. In the eye the lateral displace- 
ment of the nodal-ray is so slight, the two nodal-points being separated 
by an interval of only .37 millimetre, that it would escape observation 
in a diagrammatic drawing of the natural size. For practical pur- 
poses, therefore, we may regard the eye as having a single nodal-point. 
By calculation it is shown that this point lies very near the posterior 
surface of the crystalline lens, about 7 millimetres behind the cornea. 

Inversion of the Image. — It is apparent from Fig. 11 that the retinal 

Fig. 11. 




image is always inverted with respect to the object of vision. The 
mind, however, takes no cognizance of this inversion, since it possesses 
the power of external projection, so that we see, not the image, but the 
object in its true position. This power has doubtless been derived 
through association with the sense of touch. We have learned that a 
stimulus conveyed to the brain from the upper part of the retina pro- 
ceeds from an object situated below the eye, and vice versa, and that a 
stimulus on the temporal side of the retina must proceed from an object 
on the nasal side of the eye, and vice versa. 

Aberration. — We have assumed that all the rays proceeding from a 
point are united in its conjugate focus after refraction at a spherical 



36 THE EYE AND NERVOUS SYSTEM 

surface. This is true only of small pencils of light whose peripheral 
rays are not much removed from the optic axis. It is found by calcu- 
lation and experiment that the peripheral rays are relatively too strongly 
refracted, and consequently meet the axis sooner than the more central 
rays. This constitutes spherical aberration; it is apparent that the 
effect of aberration is to mar the sharpness of images. The refracting 
surfaces of the eye, though not strictly spherical, may practically be so 
regarded, at least at their central portions, which alone are normally 
concerned in refraction, since the iris prevents the more peripheral 
rays from reaching the retina. 

In certain cases, especially in conical cornea, the peripheral refrac- 
tion is relatively insufficient, the focal distance for peripheral rays 
being longer than that for the more central rays. This is called nega- 
tive aberration. 

There is still another kind of aberration, which is due to the fact 
that the various colors are not equally refracted, violet being the color 
of greatest and red that of least refraction. This is chromatic aberra- 
tion. 

Aberration does not ordinarily interfere with vision, partly because 
the iris cuts off the more peripheral rays, and partly because the retina 
is so constructed that a well-defined visual impression is conveyed to 
the brain only when the stimulus falls upon the macula lutea, which is 
so situated as to receive images only from points lying near the optic 
axis of the eye. While the peripheral portion of the retina is very 
useful in extending the field of vision, it is only the macula and more 
especially the fovea that is directly concerned in distinct vision. 

Angles Alpha and Gamma. — In addition to aberration, the eye is sub- 
ject to certain other imperfections. The various surfaces are not per- 
fectly centred on a single line or axis, as in a perfectly constructed 
optical apparatus, and the fovea does not lie exactly on the line which 
passes through the middle of the cornea and the centre of rotation of 
the eye, — the line which most nearly represents a common optic axis 
of the various surfaces. Since the image must fall upon the fovea in 
direct vision, it is apparent that the line connecting the point of most 
acute vision with its image on the fovea and passing through the nodal- 
point of the eye must be obliquely inclined to the axis ; that is, the axis 
of direct vision is a secondary axis. This line is called the line of 
vision. The angle which the line of vision makes with the optic axis at 
the nodal-point is called gamma. The angular distance of the fovea 
from the optic axis usually ranges from 2° to 4°, chiefly in the hori- 
zontal meridian, the fovea being usually displaced temporally as regards 



STKUCTURE AND OPTICAL PROPERTIES 37 

the optic axis. When this is the case it is apparent that the visual line 
must intersect the cornea on the nasal side of the optic axis. Further- 
more, it is found by keratometric examination that the cornea is approx- 
imately a segment of the small end of an ellipsoid, and that the corneal 
summit, or point where the major axis of the ellipsoid cuts the cornea, 
usually lies slightly on the temporal side of the optic axis. The angle 
which the major axis of the cornea makes with the visual line is called 
alpha. 

Accommodation. — We have learned that as an object approaches a 
refracting surface the conjugate image on the opposite side of the surface 
recedes from the surface. Hence if the eye is of such length that light 
from a distant object is focused on the retina, it is evident that light 
from a near object must impinge upon the retina before the rays have 
been united in a focus, unless the refractive power of the eye can be 
suitably increased. The eye does possess the property (except in old 
age) of increasing its refractive power. It accomplishes this by an 
increase in the convexity of the crystalline lens, chiefly of the anterior 
surface. This power which the eye possesses of varying its refractive 
power to adapt itself for vision at different distances is called accommo- 
dation. So long as the object of vision is distant more than about 6 
metres (20 feet) no accommodation is required; that is, for all dis- 
tances not less than this limit the posterior conjugate focus coincides 
practically with the principal focus ; or all distances nc x less than 6 
metres may be regarded as infinite in our study of the refraction of 
the eye. 

Accommodation is effected by contraction of the ciliary muscle as 
the result of reflex stimulation received by the accommodation centre 
from its connection with the optic nerve, probably through the corpora 
quadrigemina. The accommodation centre constitutes the anterior por- 
tion of the nucleus of the third nerve. From this centre an efferent 
impulse is transmitted through the ciliary ganglion to the ciliary 
muscle, and at the same time an impulse is sent to the sphincter pupillaa, 
so that contraction of the pupil takes place simultaneously with accom- 
modation. 

There are two theories as to the manner in which contraction of 
the ciliary muscle produces an increase of convexity of the crystalline 
lens. According to Helmholtz's theory, which until recently was uni- 
versally accepted, contraction of this muscle draws forward the anterior 
part of the chorioid and reduces the circumference of the ciliary muscle 
with a resultant relaxation of the suspensory ligament of the lens. The 
latter then by the inherent property of soft matter inclosed in a capsule 



38 THE EYE AKD NERVOUS SYSTEM 

and subjected to a uniform outside pressure assumes a more spheroidal 
shape with a marked increase of convexity of its anterior surface. 

Tscherning, on the other hand, has endeavored to prove that con- 
traction of the ciliary muscle causes (through the meridional fibres) an 
increased tension upon the suspensory ligament, and that because of 
the resistance of the nucleus of the lens there is a resulting increase of 
curvature at the axial part of the lens with a diminution of curvature 
at the periphery. Since this theory requires the assumption of a firm 
nucleus, it would seem untenable, for we know that no such resistant 
nucleus exists in childhood, — the period of greatest accommodative 
activity. 

Range of Accommodation. — The nearest point for which an eye can 
adapt itself is called the near-point of the eye, and the distance through 
which distinct vision is possible is called the range or amplitude of 
accommodation. In the normal eye this embraces the whole region 
from infinity to the near-point. The farthest point of distinct vision is 
called the jar-point of the eye. 

Measurement of Accommodation. — Accommodation may be measured 
by the convex lens which, when placed in front of and as near the eye 
as possible, enables light from a point to be focused on the retina with- 
out the exercise of accommodation. This lens is the equivalent of the 
accommodation which must be exercised to enable the light from the 

Fig. 12. 




same point to be focused on the retina without the lens. Thus if E, Fig. 
12, represents an eye adapted for an infinite distance, parallel rays 
will be focused on its retina, and in order that rays from a near-point, 
P, may be focused on the retina, the eye must exercise accommodation, 
or else a convex lens, L, whose focal length is LP, must be placed before 
the eye. Since P is the anterior principal focus of the lens, rays from 
P will be parallel after passing through the lens, and will, consequently, 
be focused at R on the retina of the eye, which is adapted for parallel 
rays. Hence the lens whose focal length is LP is the equivalent of the 
accommodation which must be exercised by a normal eye in order that 
rays from P may be focused on the retina. If P is the nearest point 



STRUCTURE AXD OPTICAL PROPEKTIES 39 

for which the eye can accommodate, the lens whose focal length is LP 
is the lens-equivalent of the accommodative power of the eye. 2 

Presbyopia. — As the lens hardens with increase of age there is a 
gradual diminution of accommodative power. The following table 
(Donders) gives the average accommodative power at different ages: 

Age 10 15 20 25 30 35 40 45 50 55 60 65 70 

Diopters 14 12 10 8.5 7 5.5 4.5 3.5 2.5 1.75 1 0.75 0.25 

It is shown in this table that at 4:5 years of age the amplitude is 
3.5 D. ? which represents in a normal or emmetropic eye (adapted for 
an infinite distance without accommodation) the power of focusing 
on the retina rays from all objects at or beyond a distance of ~ metre 
(9 inches). The image of an object nearer than this limit will be 
blurred. In order that a very small object, such as fine printed matter, 
may be clearly seen it must be held near the eye, so as to form on the 
retina an image large enough to be deciphered, and when the focusing 
power does not afford a clear image at a distance of 22 centimetres, or 
9 inches, the reading of very small print becomes difficult. Furthermore, 
it is not possible to use continuously all the accommodative power. It 
is found that one can use for a prolonged period only about two-thirds of 
the total amplitude ; when he has not one-third in reserve great fatigue 
(asthenopia) quickly ensues. Hence, in order that any one may use 
his eyes continuously for work at a distance of one-third of a metre 
(33 centimetres, or 13 inches) he must have a total accommodative 
power of 4:.o D. — 3 D. for use and 1.5 D. in reserve. When the am- 
plitude falls below this amount the condition is called presbyopia 
(old sight). Presbyopia is, therefore, that condition in which, from 
physiological sclerosis of the lens, the accommodative power is less than 
4.5 D. It is shown in the table that this condition makes its onset 
about the fortieth year. Usually, however, artificial assistance is not 
required before the forty-fifth year. At this age a convex spherical 
lens of 1 D. generally affords the necessary assistance. The probable 
strength of lens required to overcome the presbyopia at different ages is 
^iven in the f ollowin°; table : 



Age 


45 


50 


55 


60 


65 


70 


75 


Diopters 


1 


o 


2.75 


3.25 


3.5 


3.5 


3.5 



1 The power of a lens is inversely proportional to its focal length, which is the 
distance from the lens of the focus for parallel rays : the shorter the focal length, 
the greater is the refractive power of the lens. The unit of lens measure in oph- 
thalmology is the Diopter, which expresses the power of a lens whose focal length 
is one metre. Similarly. 2 D. is the power of a lens whose focal length is % metro ; 
3 D. is the power of a lens whose focal length is 1 ;; metre, etc-. 



40 THE EYE AND NERVOUS SYSTEM 

Obviously this table serves only as a guide. It is not to be strictly 
followed in the selection of working-glasses. The determination of the 
latter depends not only upon the distance at which work is most con- 
veniently done, but also upon the refractive condition of the eye ; that 
is, the strength of the working-lens depends upon the position of the far- 
point, or point of adaptation in relaxation of the accommodation. In 
hyperopia, as will be subsequently shown, a convex lens is required 
to adapt the eye for distant vision without accommodation. It is evi- 
dent, therefore, that in the selection of a glass for near work the correc- 
tion of the hyperopia must be added to that of the presbyopia. In 
myopia, on the other hand, a concave lens is required to adapt the eye 
for distant vision, and in this case the difference between the distance- 
glass and the presbyopic correction represents the strength of lens 
required for near work. 

ANOMALIES OF REFRACTION. 

In emmetropia, the typically normal optical condition, parallel rays 
are focused on the retina without accommodation ; that is, the eye is 
adapted for distant vision, and by the exercise of accommodation it is 
adapted for near objects. This is the most favorable condition for the 
varying requirements of civilized life, but since it necessitates an exact 
correspondence between the refractive power of the eye and the length 
of the eyeball, emmetropia, as determined by refined methods of meas- 
urement, is the exception rather than the rule. When the posterior prin- 
cipal focus does not fall upon the retina in relaxation of the accommoda- 
tion the eye is said to be ametropic or to have an error or anomaly of 
refraction. 

There are three kinds of ametropia : hyperopia or hypermetropia, 
in which the posterior principal focus lies behind the retina; myopia, 
in which the focus lies in front of the retina ; and astigmia, 3 in which, 
owing to asymmetry or irregularity of one or more of the refracting 
surfaces or to heterogeneity of structure, the rays are not brought to 
a focus at any point. 

Hyperopia. — Hyperopia may be due to any one of three causes: 
(1) deficient curvature of the cornea or lens (curvature hyperopia) ; 

3 The writer has pledged his support to the endeavor now being made by Dr. 
Swan Burnett and others to replace the commonly employed word astigmatism by 
the more euphonious and correct word here used. The impropriety of the former 
word has long been known. The argument in favor of making a change at this 
time is ably presented by Dr. Burnett in the American Journal of Ophthalmology, 
December, 1003. 



STRUCTURE AND OPTICAL PROPERTIES 41 

(2) abnormal refractive index, such that the total refractive power of 
the eye is too low (index hyperopia — very rare) ; and (3) abnormally 
short antero-posterior diameter of the eyeball (axial hyperopia). By 
means of many measurements which have been made it has been proved 
that axial hyperopia is by far the most common variety. 

In hyperopia the refractive power of the eye in relaxation of the 
accommodation is insufficient to focus rays from any point, distant or 
near, on the retina (Fig. 13). The deficiency of refraction may be 

Fig. 13. 




overcome by means of a convex spherical lens placed in front of the 
eye. This is illustrated in Fig. 14, in which parallel rays from a dis- 
tant point, 0, would be focused by the hyperopic eye, E, at some point 
behind the retina, but with the aid of the lens, L, the rays are focused 
on the retina, R. It is apparent from this diagram that LP is the 
focal length of the lens, L, which corrects the hyperopia, and that this 
focal length increases as the lens is moved farther from the eye, since 
P is a fixed point, conjugate to the retina. That lens which, when 
placed as near as possible to the eye (the position in which spectacles 
are worn), enables rays from a distant point to be focused on the retina 
is taken as the measure of the hyperopia. Thus, if LP is 1 metre there 
is hyperopia of 1 D. ; if LP is J metre there is hyperopia of 2 D., etc. 



In the study of accommodation we learned that the eye is able to 
increase its refractive power by increasing the convexity of the crystal- 
line lens. In emmetropia this power is called into play only in near 
vision, but in hyperopia, when uncorrected by a lens, accommodation 
must be exercised to render distant vision distinct, and in near vision 
the same degree of accommodation which would be required of a nor- 
mal eye must be used in addition to that required for distant vision. It 



42 THE EYE AND NEKVOUS SYSTEM 






is apparent, therefore, that the unaided hyperopia eye has an abnormal 
task imposed upon it, both in distant and in near vision. In childhood 
when the accommodative power is at its maximum a moderate degree 
of hyperopia is very often not attended by any disturbance, the accom- 
modation being ample for near as well as for distant vision, but when 
with advancing age the accommodative power diminishes, the tax im- 
posed upon the ciliary muscle gives rise to various symptoms of nerve- 
exhaustion ( asthenopia ) . 

So easy is accommodation in childhood that it is usually not possi- 
ble for a hyperope at this period of life to relax all of his accommoda- 
tion when using his eyes. Hence a portion of the hyperopia will be 
masked by accommodative action, and it is only by the employment of 
a cycloplegic or by examination in a dark room that the full extent 
of the hyperopia can be ascertained. That portion of the hyperopia 
which is masked by accommodation is called latent hyperopia. Hyper- 
opia which exists in excess of what is latent 'is called manifest; that is, 
manifest hyperopia is measured by the strongest convex lens which does 
not impair distant vision. The sum of the latent and manifest hyper- 
opia constitutes the total hyperopia. In childhood hyperopia of a low 
or moderate degree is very frequently all latent, but sooner or later, the 
age varying with the degree of hyperopia and with the physical condi- 
tion, a portion becomes manifest, and this portion continually increases 
at the expense of the latent hyperopia. 

There is, further, a division of manifest hyperopia into that which 
can be overcome by accommodative action to render distant vision dis- 
tinct {facultative hyperopia), and that which cannot be so overcome 
(absolute hyperopia). In old age the total hyperopia is absolute, so 
that distant vision can be made distinct only by the help of a convex 
lens. In near work an absolute hyperope would require the appropriate 
presbyopic correction added to the correction of the hyperopia. Thus 
in hyperopia of 2D., a lens of 5 D. would be required for reading at 
a distance of J metre (13 inches). 

Hyperopia is the normal condition in infancy, but with the increase 
of the antero-posterior diameter of the eye which takes place in the 
process of growth, hyperopia is succeeded by emmetropia or myopia 
prior to adult life in about one-half of all persons in civilized countries. 
Among savages hyperopia is the rule at all ages. 

It is apparent, therefore, that hyperopia (except in the higher 
grades) is, like presbyopia, a physiological condition ; yet, owing to the 
great preponderance of near-work which results from the requirements 
of civilization, it is in most cases necessary to correct all or a portion 



STRUCTURE AKD OPTICAL PROPERTIES 43 

of the defect before the hyperope has advanced far in adult life. In 
fact, even a moderate hyperopia may require correction in childhood to 
relieve the strain which is imposed by the unnatural near-work incident 
to school life. 

In the treatment of hyperopia a number of questions arise, the most 
important of which may be summarized as follows: (1) As to when 
it is advisable to correct the defect; (2) as to the portion of the defect 
to be corrected ; and ( 3 ) as to whether the glasses should be worn con- 
stantly or for near-work only. 

As to the first question, hyperopia does not require correction unless 
it gives rise to some physical disturbance, as inability to see clearly, 
asthenopia, headache, or convergent strabismus ; but since few persons 
consult a physician except when suffering from physical disability, it is 
apparent that almost all cases of hyperopia which are brought to the 
notice of the physician require correction. 

As to the portion of error to be corrected, it is not possible to give a 
fixed rule. Usually in childhood the lens should not exceed one-half or 
two-thirds of the total error as determined with the eye under the in- 
fluence of a cycloplegic. The chief exception to this rule is when the 
hyperopia is complicated with convergent strabismus, when all or nearly 
.all the error should be corrected. In adults correction of the greater part 
•of the hyperopia will usually be tolerated. 

Still less is it possible to answer the third question by a fixed rule. 
In general, children who suffer from asthenopia resulting from a low 
grade of hyperopia should wear correcting glasses during school and 
.study hours only; but when the error reaches 2.5 D. or 3D. it is 
usually found that the ciliary muscle will not adapt itself to the use of 
glasses unless the latter are worn constantly. Similarly in strabismus 
relief is not to be expected unless the glasses are worn constantly. In 
.adult hyperopes who suffer from nerve-strain, as the result of prolonged 
near-work, the same general rule is applicable; that is, the glasses may 
De worn for near-work only, if the error is slight, and especially if it is 
latent, but for higher grades of the defect the glasses must be worn 
constantly. 

Myopia. — Myopia is divisible into curvature myopia, index myopia, 
and axial myopia. Curvature myopia results from keratoconus or conical 
cornea, or from increased convexity of the lens in partial dislocation 
(when released from traction by the suspensory ligament). Myopia 
also occurs not infrequently as a premonitory sign of senile cataract, and 
in diabetes. This may be due either to increase of convexity of the 
lens from swelling or from increase of refractive index of the nuclear 



44 



THE EYE AND NEKVOUS SYSTEM 






portion of the lens. With these exceptions myopia is almost always 
due to excessive length of the antero-posterior diameter of the eyeball. 
In myopia, whatever its cause, the refractive power of the eye is 
too great in proportion to the axial length, and consequently rays from 
a distant object (parallel rays) will be united in a focus before they 
reach the retina, and the image of a distant object as formed on the 
retina will be blurred (Fig. 15). The predominant symptom of 

Fig. 15. 




myopia is, therefore, the inability to see distant objects clearly, and 
since only near objects are clearly distinguished, the affection is popu- 
larly called near-si glit edness. 

An object situated at P (Eig. 16), which is conjugate to the retina, 
and which is the far-point of the eye, can be clearly seen without any 
accommodation, and an object nearer the eye than this point can be 
seen with less accommodation than would be required by a normal eye, 
whose far-point is at infinity. This is not, however, as it might appear, 
always an advantage in near-work, for it not infrequently leads to 
severe strain on the convergence centres, the nervous mechanism being 
so constituted that exercise of accommodation is required as an incentive 

Fig. 16. 




for the proper convergence of the visual lines of the two eyes, so that the 
image may in each case fall upon the fovea. Furthermore, when the 
myopia exceeds 3.5 D. or 4 D., the farthest point of distinct vision is 
so near the eyes that the amount of convergence required is too great to 
be comfortably maintained ; in uncorrected high myopia, vision is per- 
formed with one eye, while the other assumes a position of relative or 
absolute divergence (divergent strabismus). 



STKUCTUBE AOT3 OPTICAL PKOPEETIES 45 

Axial myopia, which, as previously stated, is the typical form of 
myopia, is divisible into two classes. The first class embraces those 
cases in which the eyes are hyperopic prior to the beginning of school 
life. In the natural course of growth there occurs an enlargement of 
the eyes with consequent diminution of hyperopia, and, normally, hy- 
peropia passes into emmetropia before adult life. But in a certain pro- 
portion of cases the increase in the axial length is not arrested when 
emmetropia is reached, and a condition of myopia results. This is 
probably caused by stretching of the sclera by muscular pressure and 
traction in the convergence required in school work. This kind of 
myopia does not reach a very high degree and it ceases to advance, after 
the sclera has acquired its normal resisting power, in adult life. It is, 
therefore, called benign or school myopia. 

The second class of axial myopia is due to defective development 
of the sclera ; the myopic condition is present at an early age — prior to 
the beginning of school life. It may r>3ach a very high degree {posterior 
staphyloma), and together with the distention of the sclera there may 
occur atrophy of the chorioid, vitreous opacities, hemorrhages, or de- 
tachment of the retina, with partial or total loss of vision. This kind 
of myopia is, therefore, appropriately called malignant myopia. 

The malignant variety is fortunately much less common than the 
benign. While the latter is confined chiefly to the student classes, the 
former occurs in a relatively greater proportion among the poorly de- 
veloped lower classes. The proportion of myopes varies in different 
countries. In America statistics have shown a proportion of about 
25 per cent, among college students, while in Germany the proportion 
among the same class of persons reaches 50 per cent. 

As in hyperopia the deficiency of refraction is overcome by the 
convergent action of a convex lens, so in myopia the excess of refrac- 
tion may be overcome by the divergent action of a concave lens. Thus if 
L (Pig. 16) is a concave lens, the parallel rays which meet it are ren- 
dered divergent so that they appear, after passing through the lens, to 
proceed from the point P. Hence if P is conjugate to the retina of the 
myopic eye E, it is apparent that parallel rays which are made to pass 
through the lens will enter the eye E as if coming from P, and will con- 
sequently be focused on the retina. An appropriate concave lens will, 
therefore, make distant objects appear distinct to the myopic eye. The 
eye may then see near objects with the aid of accommodation, or the 
lens may be removed in near vision. 

It is apparent from Pig. 16 that parallel incident rays will never 
be united in a focus as the result of refraction by a concave lens, but P 



46 THE EYE AKD NEKVOUS SYSTEM 

is the point on the axis through which, after refraction, the rays appear 
to pass. The point P is, therefore, the virtual focus (for parallel rays) 
of the concave lens, in contradistinction to the real focus of the convex 
lens. The focal length of the lens L is LP; it is expressed in diopters,. 
as was explained for the convex lens ; but since P lies on the left of the 
lens, instead of on the right as in the convex lens, the focal length is 
regarded as negative, and the refractive value is expressed by the minus 
(-) sign, which is used before the dioptric number. 

The degree of myopia is measured by the focal length or dioptric 
power of T;he lens, which, when placed as near as possible to the eye, 
focuses parallel rays on the retina. Thus if LP is 1 metre, the eye has 
1 D. of myopia ; if LP is J metre, the eye has 3 D. of myopia, etc. 

In the adaptation of concave lenses to the correction of myopia the 
general rules differ somewhat from those given for the correction of 
hyperopia. Myopia occurring in young children should be corrected at 
as early an age as is compatible with the wearing of spectacles, not only 
for the beneficial effect in retarding the advance of the myopia (prob- 
ably by relieving the eyes of the necessity of excessive convergence), but 
also for the purpose of placing the child under favorable circumstances 
in cultivating his powers of observation. All, or all but a small frac- 
tion, of the myopia should be corrected, and the glasses should be worn 
both for distance and near-work. In the case of adults, however, when 
the myopia has not been corrected in childhood, the ciliary muscle is 
poorly developed, and the full amplitude of accommodation is not 
attained. In such cases it often happens that the glasses which 
are suitable for distance will not be tolerated for near-work; for the 
latter a weaker concave lens, or no lens, or a weak convex lens may 
be required, according to the degree of myopia and the age of the 
individual. 

In the highest • grades of myopia (exceeding 15 D.) the myopia 
may be overcome or greatly reduced by extraction of the crystalline 
lens. 

Of no less importance than the correction of myopia is its 
prophylaxis by the inculcation of proper hygienic principles, such as 
the avoidance of close application of the eyes at a very early age, the 
necessity of good illumination of school rooms, and of large and clear 
print for young children. 

Astigmia. — Astigmia is that condition in which rays proceeding 
from a point are not united in a focus at another point after refraction. 
There are two kinds of astigmia of .the eye: (1) That which is due to 
asymmetrical, but regular, curvature or oblique position of the cornea 



STRUCTURE AXD OPTICAL PROPERTIES 47 

or lens; 4 and (2) that which is due to unevenness or irregularity of 
one or more of the surfaces, or to heterogeneity of composition of the 
crystalline lens. The first kind constitutes regular and the second 
irregular astigmia. 

Astigmia exists, to a slight extent, as a physiological imperfection 
in all eyes. The curvature of the cornea is, as a rule, somewhat greater 
in the vertical than in the horizontal meridian. On the other hand, the 
crystalline lens presents the opposite condition, for, on account of a 
slightly oblique position, its meridian of greatest refraction is usually 
horizontal. These two defects of curvature tend to neutralize each 
other, but as a rule the vertical refraction predominates, thus giving rise 
to a slight degree of regular astigmia. Furthermore, the normal crys- 
talline lens is not free from heterogeneity of structure, for, being com- 
posed of a number of segments, and each segment of a number of 
fibres, its refraction is considerably marred by irregularity, which is 
the chief cause of the normal irregular astigmia. 

Regular astigima, exceeding the amount which may be regarded as 
normal or physiological, may be due to excess of vertical over the hori- 
zontal curvature {astigmia with the rule); or to excess of horizontal 
curvature {astigmia against the rule); or to asymmetry in which the 
meridians of greatest and least curvature (these being at right angles 
to each other) are neither vertical nor horizontal {oblique astigmia). 
As may be inferred from the normal asymmetry of the cornea and lens, 
astigmia with the rule is more commonly the result of corneal asym- 
metry {corneal astigmia), while astigmia against the rule is more com- 
monly due to asymmetry of the lens {lenticular astigmia) ; but this is 
by no means always the case, for quite often the meridian of greatest 
corneal curvature is horizontal, thus giving rise to astigmia against the 
rule. This is notably so after cataract operations, since the section of 
the cornea leaves a resulting diminished curvature in the vertical direc- 
tion without materially altering the curvature of the horizontal 
meridian. 

Regular astigmia, since it results from a regular asymmetry of 
curvature, in which the meridians of greatest and least curvature (the 
principal meridians) are at right angles to each other, may be overcome 
by a cylindrical lens, which has the property of refracting rays in the 

* The astigmia which results from obliquity of the cornea or lens with refer- 
ence to the optic axis is usually classed as regular since the defect of vision result- 
ing therefrom can be improved by a cylindrical lens; but from a mathematical 
point of view the condition differs somewhat from that which results from asym- 
metrical curvature. 



48 THE EYE AM) NERVOUS SYSTEM 

meridian of its curvature without altering their direction in the plane 
of the axis of the cylinder. Hence if the refraction of an eye is such 
that rays proceeding from a distant point are, in the vertical meridian, 
focused on the retina, while in the horizontal meridian of less curva- 
ture the rays are intercepted by the retina before they have reached 
their focus, a suitable convex cylindrical lens, having its refracting me- 
ridian horizontal (its axis vertical), will so add to the refraction in the 
horizontal meridian that the rays will be focused on the retina in this 
as well as in the vertical meridian; in other words, the rays will be 
focused in a point on the retina and the astigmia will be overcome. 
When, as in this case, the eye is emmetropic in one meridian and hy- 
peropic in the other, the condition is called simple hyperopic astigmia. 

If with the same asymmetry of curvature the eyeball is slightly 
larger than in the former case, so that the horizontal focus falls on the 
retina while the vertical focus is in front of the retina, the condition is 
simple myopic astigmia. In this case rays will be focused in a point 
on the retina with the aid of a suitable concave cylindrical lens, axis 
horizontal, since this will throw the vertical focus back to the retina 
without affecting the rays in the emmetropic horizontal meridian. 

If both foci are behind the retina, one farther than the other (com- 
pound hyperopic astigmia), a convex spherical lens must be added to 
the appropriate cylindrical lens in order to bring the focus on the 
retina. If, on the other hand, both foci are in front of the retina (com- 
pound myopic astigmia) a concave spherical lens must be added to the 
appropriate cylinder. It may happen evidently that the retina is situ- 
ated between the two foci, one of these being in front of the retina and 
the other behind it (mixed astigmia). In this case a convex cylinder, 
such as will equalize the refraction in the^ two principal meridians and 
thus bring the rays to a focus in front of the retina, may be combined 
with a concave spherical lens of sufficient strength to throw the focus 
back upon the retina; or a concave cylinder, such as will equalize the 
refraction but throw the focus behind the retina, may be combined with 
a convex spherical lens to bring the focus forward upon the retina. 5 

3 Sphero-cylindrical lenses for the correction of compound and mixed astigmia 
have the spherical element ground upon one side of the glass and the cylindrical upon 
the other. In the correction of compound astigmia both surfaces should be convex 
or both concave, according as the condition is that of hyperopic or myopic astigmia, 
but in the correction of mixed astigmia one surface must be convex and the other 
concave. The sphero-cylinder is the common form of lens used in the correction of 
these errors, but it is possible to substitute for this form of glass a crossed- 
eylinder (one cylinder on each side of the glass, the two axes being at right 
angles ) , or a toric lens, in which the proper asymmetrical curvature is ground upon 
one face of the glass. 



STRUCTURE AND OPTICAL PROPERTIES 49 

The degree of astigmia is measured by the dioptric power (in the 
refracting meridian) of the cylindrical lens which equalizes the refrac- 
tion. The degree may vary from .12 D. (the weakest lens commonly 
made) to 15 D. or more. Almost all eyes have as much asymmetry as is 
indicated by the former limit, but the higher degrees are fortunately 
uncommon. In fact, astigmia of more than 5 D. or 6 D. is not often 
encountered. In the lower grades of asymmetry the effect upon vision 
is very slight, and in many such cases no disturbance of any kind 
results ; on the other hand, in persons who are engaged in close applica- 
tion of the eyes astigimia of .25 D. or even .12 D. may produce asthen- 
opia, which is relieved only by the use of correcting lenses. 

The treatment of regular astigmia consists in the adaptation (as will 
subsequently be explained) of the appropriate cylindrical lens. In the 
lower grades the lens-correction may be worn only in close application 
of the eyes, but in the higher grades, preferably in all reaching 1 D., 
the correction should be worn constantly. 

Irregular astigmia exceeding the physiological amount is most com- 
monly due to scar formation in the cornea or to irregular swelling of 
the crystalline lens in the premonitory stage of cataract. This kind of 
astigmia is incapable of correction. 

Anisometropia. — Since each individual is normally endowed with 
a pair of eyes, which work together in the function of vision, the rela- 
tive refractive condition of the two eyes is a matter of great importance. 
The ideal condition is evidently that in which the refraction is alike in 
the two eyes ; this is called isometropia. But more commonly an abso- 
lute correspondence of the two eyes is not found ; one eye may be emme- 
tropic and the other hyperopic, myopic, or astigmic; or both eyes may 
have the same defect, the degree being higher in one eye than in the 
other ; or one eye may have hyperopia and the other myopia. Any such 
deviation from isometropia is called anisometropia, and the special con- 
dition in which one eye is hyperopic and the other myopic is usually 
called antimetropia. While it is, in most cases, possible to detect a 
slight difference between the two eyes, it is only when the disparity is 
marked that anisometropia should be regarded as an anomaly, capable 
of giving rise to asthenopia or other visual disturbance. 

In the treatment of anisometropia the ophthalmologist endeavors to 
equalize the condition, as far as practicable, in the two eyes by giving 
to each eye its appropriate correcting lens. In childhood quite a high 
disparity may thus be overcome, but in those cases which have not been 
corrected prior to adult life the correction of the defect will, in the more 
marked cases, rarelv be tolerated. In some of these cases vision is 



50 THE EYE AND NERVOUS SYSTEM 

performed with both eyes, the clear image formed in one eye being 
fused with the blurred image formed in the other, but in very many 
cases vision is uniocular, the better eye being used, while the poorer eye 
falls into a state of relative deviation or squint. On this account it is 
of the utmost importance that anisometropia should be corrected in 
early childhood. 

Determination of Visual Acuteness and of the 
Refractive Condition of an Eye. 
The visual power is measured by the least interval which permits 
the eye to distinguish as such two bright or white points on a black 
ground. In the normal eye this minimum interval subtends an angle 
of about one minute at the nodal-point. This angle is called the mini- 
mum visual angle. For practical vision-testing it is more convenient 
to use a series of test-letters, so constructed that when they are placed 
at the proper distance, the breath of each stroke of the letter sub- 
tends the minimum visual angle at the nodal-point of the eye under- 
going examination, while each dimension of the whole letter subtends 
an angle of five minutes (Fig. 17). For determining visual acuteness 



Fig. 17. 




: "U.l 




1 ] 


1 | 




cards are furnished having printed on them letters of various sizes, the 
distance at which each letter subtends the five-minute angle being noted 
on the card. Thus, if at a distance of six metres, a person can dis- 
tinguish those letters which, as marked on the card, subtend the five- 
minute angle at this distance, the visual acuteness is normal ( V = f 
or 7 = 1); but if at this distance the smallest distinguishable letters 
are those which subtend the five-minute angle at twelve metres, the 
visual power is only x% of the normal acuteness ( V = tt) • The visual 
acuteness of any eye is therefore measured by a fraction whose numer- 
ator is the distance at which the test is conducted and whose denominator 
is the distance at which the smallest distinguishable letters subtend the 
five-minute angle. 

It not infrequently happens, especially in young persons, that the 
visual power exceeds the standard which has been accepted as the aver- 



STEUCTUEE AND OPTICAL PEOPEETIES 51 

age vision of normal eyes. We thus have the expressions V =i, or 

y — 4* 

The minimum visual angle is the basis of the most valuable method 
of determining the refractive condition of the eye. The refraction of 
the eye indicates the relation between the position of the retina and pos- 
terior focus for parallel rays, when the accommodation is relaxed. 6 
Hence in determining the refraction the test-letters must be at such a 
distance that the rays proceeding therefrom may be regarded as parallel ; 
that is, the letters must be five or preferably six metres (20 feet) from 
the eye undergoing examination. With the letters at this distance nor- 
mal vision is possible only in emmetropia or in hyperopia with use of 
accommodation, since full visual acuteness necessitates an accurate focus 
of the image on the retina. To decide between these two conditions Ave 
place a weak convex spherical lens before the eye under examination 
(the other eye being excluded from vision by an opaque disk) ; if this 
lens does not render vision worse (hazy) accommodation has been re- 
placed by the convergent action of the lens. By trying successively 
stronger lenses we find the strongest lens which does not impair vision* 
This measures the manifest hyperopia. In old persons it also measures 
the total hyperopia, but in children and young adults a certain portion of 
the hyperopia is, as we have learned, latent. In fact the whole of the 
hyperopia may be latent, so that the weakest convex lens will make vision 
worse. When latent hyperopia cannot be otherwise excluded a cyclo- 
plegic must be instilled into the conjunctival sac, and the examination 
made while the eye is under the influence of the drug. If under this 
condition vision remains normal, the eye is emmetropic; but if the 
vision, which before was normal, falls below normal during cycloplegia, 
the convex spherical lens which affords maximum vision measures the 
total hyperopia. 

Homatropin-hydrobromate is the most suitable cycloplegic for gen- 
eral use ; one drop of a one-and-one-half per cent, solution instilled at 
ten-minute intervals until six applications have been made will, except 
in rare instances, produce complete paralysis of the accommodation in 
one hour after the last instillation. The effect of the drug passes off in 
about twenty-four hours. In young children and in other persons in 
whom a prolonged enforced rest of the accommodation is desirable 
atropin may be used. To insure complete paralysis, in execessive accom- 
modative activity, one drop of a one per cent, solution of atropin-sul- 
phate may be instilled three times a day for several days, though usually 

6 This is the static refraction, in contradistinction to the di/iiamic refraction, 
which refers to the accommodative power. 



52 THE EYE AND NERVOUS SYSTEM 

complete paralysis occurs in one and one-half hours after the first in- 
stillation. The full accommodative activity is not restored until about 
two weeks after the last instillation. 

The use of a cycloplegic is contraindicated when there is any ten- 
dency to glaucoma; but as this affection seldom occurs in young per- 
sons, who alone require a cycloplegic for the determination of refraction, 
there is ordinarily no danger in the use of a cycloplegic for this purpose. 

If vision is less than normal at the first examination (without cyclo- 
plegia), the defect of vision is due, if to any error of refraction, either 
to hyperopia without sufficient accommodation to overcome it, or to 
myopia, or to astigmia. The first of these conditions is determined by 
the improvement effected by placing a convex spherical lens before the 
eye. On the other hand, the presence of myopia is revealed by the fact 
that vision is rendered worse by a convex lens and improved by a con- 
cave lens. But while in hyperopia the strongest acceptable lens measures 
the ametropia, in myopia the weakest lens which affords maximum 
vision must be selected, for if a stronger lens is taken, normal vision 
may still be possible with the aid of accommodation. In fact, so active 
is accommodation in young subjects that overaction of the ciliary muscle 
(spasm of accommodation) sometimes gives rise to an apparent myopia, 
whereas the true condition is that of emmetropia or hyperopia. When 
there is any reason for suspecting an overaction of the ciliary muscle 
the refractive condition should be determined while the eye is under the 
influence of a cycloplegic. In fact it is better in most cases to use a 
cycloplegic for the determination of refraction in persons under thirty 
years of age, since by this means only can the static refraction be de- 
termined with absolute certainty. 

When neither a convex nor a concave spherical lens brings the vision 
up to the normal standard, we suspect the presence of astigmia. If this 
is regular it may be corrected by a cylindrical lens. If a spherical lens 
has been found which improves vision, this should remain before the 
eye in the trial frame in conducting the examination for the determina- 
tion of the existence of astigmia. In the latter procedure we place a 
weak convex cylindrical lens before the eye ; and, since more commonly 
the eye has its greatest curvature in the vertical meridian, we place the 
axis of the lens in this meridian. If in this position it does not improve 
vision, we rotate the lens to ascertain whether in any other position it 
effects an improvement. 7 If we thus succeed in finding a position in 
which the lens improves vision, we try stronger lenses until we find 

T The position of a cylindrical lens is registered in accordance with the direc- 
tion of its axis, as indicated by the angular marking on the trial frame. 



STEUCTUEE AND OPTICAL PEOPEETIES 53 

that lens which gives normal vision. If the weak convex cylinder is not 
accepted in any meridian, we next try a concave cylinder. In this case 
we first place the axis in the horizontal meridian, since the probability is 
that the vertical curvature is excessive. We then proceed as with convex 
cylinders until we find the position and the lens which give best vision. 
If we have difficulty in determining the directions of the principal me- 
ridians by the foregoing method, we may make use of the cloclc-face 
chart (Fig. 18), which consists of a series of radiating lines, numbered 




in the same manner as a clock face. If (after correction of any exist- 
ing hyperopia or myopia) one group of these lines appears very dis- 
tinct while the other lines, and especially the group at right angles to 
the group of greatest distinctness, are blurred, the eye is astigmic, and 
the lines of greatest distinctness lie in the meridian which requires 
correction. 8 Hence the axis of the cylindrical lens should be placed in 
the direction of the most blurred lines. If a convex lens having its 
axis in this direction does not improve vision, we try a concave lens 
with its axis in the same direction. The lens which equalizes all the 
lines and at the same time affords normal vision is the correction of 
the astigmia. 

While simple refractive errors in eyes having normal visual acute- 
ness may be easily measured by the method of examination just de- 
scribed, those cases in which hyperopia or myopia is complicated by 
astigmia, and especially in eyes having imperfect visual acuteness, much 
time may be saved and greater accuracy secured by the aid of objective 

8 If, for instance, the eye is emmetropic in the horizontal meridian, but not so 
in the vertical meridian, a distant point will have its horizontal focus on the 
retina, while in the vertical meridian the rays will be diffused on the retina, that 
is, the image of a distant point will be a vertical line; therefore since every point 
of a vertical line will have a corresponding vertical line as its image, the image 
will be a sharply defined line. But if the line is horizontal instead of vertical, 
that is, if the line lies in the emmetropic meridian, every point of the horizontal 
line will have a short vertical diffusion line as its image, and the image of the 
whole line will be diffused and blurred. 



54 THE EYE AND KEKVOUS SYSTEM 

methods of examination. The most valuable of these methods are 
ophthalmoscopy , skiascopy, and keratometry. 

The refractive condition may be determined, with a degree of accu- 
racy varying with the experience and skill of the examiner, by direct 
ophthalmoscopy. If the examiner is emmetropic (or has his ametropia 
corrected by a lens) the hyperopia of the examined eye is measured by 
the strongest convex ophthalmoscopic lens with which the small retinal 
vessels can be distinctly seen ; while myopia is measured by the weakest 
concave lens with which these vessels are distinctly seen. Astigmia is 
measured by the difference between the lens which corrects the ame- 
tropia in one principal meridian (as determined by distinctness of the 
retinal vessels at right angles to this meridian) and the lens which 
corrects the ametropia in the other principal meridian. The accurate 
estimation of astigmia in this way is very difficult; in fact, it is not 
usually attempted, since the astigmia can be much more easily de- 
termined by skiascopy. 

Skiascopy consists in the estimation of the refractive condition from 
the movement of the shadow which passes across the pupil of the ex- 
amined eye when the illuminating mirror is rotated. The movement of 
this shadow varies with the position of the observer (who looks through 
the sight hole of the mirror), with reference to the far-point of the 
examined eye. If the examiner is at this point there is no perceptible 
movement of the shadow, but, as the mirror is shifted, the whole pupil 
is simultaneously enshrouded in darkness. If the examiner is nearer 
the eye than the far-point, he sees a shadow move across the pupil in the 
direction in which he rotates the mirror, the rate of movement diminish- 
ing as he departs from the far-point. If, on the other hand, he is 
farther from the eye than the far-point, he sees the shadow move in the 
opposite direction to the mirror rotation. 9 The explanation of this 
change of movement is, found in the fact, that the rays proceeding from 
any point of the retina meet at the far-point of the eye and have their 
relative position reversed in passing this point. On this account the 
far-point is called the point of reversal of the eye. We know that in 
emmetropia the far-point (the anterior conjugate to the retina) is at 
infinity, and in hyperopia it is negative or behind the retina ; hence it is 
only in myopia that the examiner can place himself at the point of 
reversal. If, for instance, he finds that there is no perceptible shadow 
at a distance of one metre, while the shadow moves opposite to the 

• 9 The movements as described are those that take place with a plane mirror ; 
if a concave mirror is used, the direction of the movement is in all cases opposite 
to that described for the plane mirror. 



STRUCTURE AXD OPTICAL PROPERTIES 55 

mirror when lie moves to a greater distance, and in the same direction 
as the mirror when he moves nearer the eye than the one-metre limit, 
he knows that the eye has 1 D. of myopia, since the degree of myopia is 
measured by the distance of the far-point from the eye (Eig. 16). But 
if as the examiner moves away from the eye the shadow continues to 
move in the same direction as the mirror, he may place a convex spherical 
lens before the eye so as to render it artificially myopic. Thus, if with 
a convex lens of 1 D. the shadow movement is annulled at a distance of 
1 metre, the eye must be emmetropic, since one diopter of myopia is 
produced by a lens of 1 D. But if it requires a lens of 2 D. to bring the 
point of reversal to the one-metre distance, the eye must have 1 D. of 
hyperopia. In other words, if the examiner places himself at a dis- 
tance of one metre from the eye, the hyperopia is always 1 D. less than 
the dioptric power of the lens which annuls the shadow-movement. In 
myopia of more than 1 D. the examiner may approach the eye until he 
reaches the point of reversal and thus measure the distance of the far- 
point directly, or he may (as is generally preferable) remain at the one- 
metre position and place before the eye such a concave lens as will bring 
the point of reversal to this position. The lens which effects this, neu- 
tralizes all but 1 D. of the existing myopia ; that is, the myopia is 1 D. 
greater than the dioptric power of the lens which annuls the shadow- 
movement. 

If in the application of skiascopy we find (either with or without a 
spherical lens) no shadow-movement in one meridian, while a shadow 
appears to move across the pupil in the meridian at right angles to this, 
the existence of astigmia is demonstrated, and the meridian in which 
there is no movement is one principal meridian, while the other is the 
meridian in which the shadow appears to move. The degree of astigmia 
is then measured by the cylindrical lens, having its axis in the meridian 
of no movement, which, either alone or together with a spherical lens, 
.annuls the movement in all meridians. 

Keratometry, or ophthalmometry, consists in the measurement, by 
means of images reflected at the anterior surface of the cornea, of the 
curvature of this surface, and the deduction therefrom of the corneal 
asymmetry in terms of the dioptric equivalent of the resulting astigmia ; 
but since the corneal astigmia is to a variable extent modified by the 
lenticular astigmia, which is not revealed by keratometry, this method 
is less valuable than skiascopy; it is, nevertheless, very useful as a 
corroborative test. 

Determination of the Accommodative Power. — The accommodative 
power may be most conveniently measured by means of small test-letters 



56 THE EYE AND NEKVOFS SYSTEM 

constructed on the five-minute-angle principle, as have been devised by 
Oliver. If the eye is emmetropic, the accommodative power is measured 
by the shortest distance from the eye at which the smallest distinguish- 
able letters can be read. If the eye is ametropic, its refractive error 
should be first corrected, and the examination then conducted as in 
emmetropia. 



CHAP TEE III. 

THE PSYCHOLOGY OF THE VISUAL ACT AND THE FOCAL 
DISEASES OF THE VISUAL COETEX. 

By CHARLES K. MILLS, M. D. 

INTRODUCTORY OUTLINE. 

The visual act may be described in general terms as that process by 
which luminosity, form or color is recognized by the organs of vision. 
Usually the act is concerned with the visual recognition of a definite 
object. The term has, however, an application to subjective visual 
processes as illustrated, for instance, by hallucinations of sight either in 
the normal or in the insane, and by the recognition of phosphenes due 
to mechanical or electrical stimulation. My concern in the present 
chapter is chiefly with the visual act as related to the cerebral cortex, and 
especially to the higher cortical areas and centres of vision. The con- 
sideration of the subject must necessarily include to a certain extent 
both the physiology and the pathology of the visual cortex. The cortical 
visual act may be simple or complex according to the simplicity or com- 
plexity of the object recognized, which may vary from a luminous point 
of weak intensity and brief duration through a series including forms 
of all grades of regularity or irregularity, colors differing in hues and 
in shades, natural objects of diverse sort, particular landscapes, persons 
with individuality of face and figure, designs originating in the arts and 
sciences, and numbers, letters and words. 

Under the influence of the recent discovery of radium and its effects, 
some tendency is shown among physicists to return to the corpuscular 
theory of light ; nevertheless the still most universally accepted doctrine 
is that which is based upon the Newtonian hypothesis of luminous trans- 
mission by waves of the ether which permeates, all space. 

Unlike sound vibrations, the direction of the waves of the vibrating 
ether is transverse to the direction of the rays of light. Only those ethe- 
real vibrations which have a velocity of between four hundred billions on 
the one hand and a little more than nine hundred billions on the other 
hand are effective in giving rise to sensations of sight. 

To others in this work are assigned the tasks of not only describing 
and explaining the anatomy and methods of action of the components 
of the eye itself, but also of many of the effects which result from the 

57 



58 THE EYE AND NERVOUS SYSTEM 

reception of light by the nervous apparatus of vision. I shall consider 
only the ultimate cerebral, and therefore psychical, effects of the light 
transmitted through the eye to the nervous system. In the retina are 
certain photo-chemical substances and a complex anatomical nervous 
structure, the chief constituents of which are known as the rods and 
cones. To and from these rods and cones come and go transmitting ner- 
vous structures which put the retinal elements in communication with 
lower and higher levels of the brain. 

A light wave starts on its journey through the ether from the lumin- 
ous object which is its source. The first step towards its becoming 
a visual impulse is taken when it decomposes the photo-chemical sub- 
stances of the retina, thus setting up vibrations in the extreme periphery 
of the end organs of this membrane. The initial psychical step is taken 
when it reaches its first stopping place in the brain cortex. 

The excitation received by the retinal substances and structures is 
conveyed by fibres of the optic nerve back to centres at the base of the 
brain, and either directly or by new relays of fibres to the visual cortex. 
Definite portions of the retina are related to equally definite portions of 
the visual centre which first received the projected retinal excitations — a 
matter which will later receive attention in more detail. The result in 
the cortical centres first receiving the impulses is a visual sensation or 
percept. Up to this point, however, an idea of the object perceived is not 
obtained. In order that this shall come to pass the cortical excitation 
which has been evoked must be transmitted to the cellular elements of 
another region of the brain surface ; in other words, from a simple sen- 
sory or percept centre to a memory centre. A memorial image is de- 
posited in the centre placed at some distance from the cell or cell-group 
in which the excitation is received. 

When such focal diseases of the visual cortex as mind-blindness, word- 
blindness and letter-blindness are considered, the fact that visual sensa- 
tions or percepts and visual concepts or appercepts are dependent upon 
separate processes and have distinct cortical habitations, will more 
clearly appear. 

One who is mind-blind or word-blind so that he may not be able to 
recognize persons or objects in their real characters, or words and letters 
in their true significance, may yet have visual sensations with their cor- 
tical correlatives, as illustrated by the fact that he can avoid or seek the 
objects which he does not recognize and that he may see the words and 
the letters composing them and yet not be able to tell what, they import, 
although he knows that they are different from the other objects in his 
field of vision. 






THE PSYCHOLOGY OF VISION 59 

LIMITS AND SUBDIVISIONS OF THE VISUAL CORTEX. 

I shall next define the limits and subdivisions of the visual cortex as 
I understand them. The visual cortex, that part of the convoluted cere- 
bral surface concerned with the function of sight, includes the entire 
occipital lobe both on its mesotentorial and its lateral aspects; also 
taking in adjoining caudal portions of the parietal and temporal lobes. 
This cortical visual field is of complex composition. It includes a pri- 
mary or lower and a secondary or higher cortical visual area. The 
higher area is extensively subdivided. The primary or lower cortical 
visual area which corresponds to the optic perception field of Wilbrand 
and Saenger 1 , is situated chiefly, but not exclusively, on the mesotento- 
rial surface of the occipital lobe ; the secondary or higher cortical visual 
area corresponding to the optic memory field of the authorities just men- 
tioned, covers a large extent of the lateral aspect of the occipital lobe, 
spreading, to some extent, over the temporal and tentorial surfaces. 

In order to understand the limits and subdivisions of the visual cor- 
tical areas, in accordance with the broad statements just made, it is 
necessary to have some idea of the views of Flechsig. 2 

His views are based upon his studies in myelinization as especially 
observed in the brain of the foetus and of the infant shortly after birth. 
He believes that. the nerve fibres connected with special cortical areas 
become medullated at certain definite periods, these myelogenetic areas 

1 Wilbrand and Saenger, Die Neurologie des Auges, vol. iii. Anatomie und 
Physiologie der Optischen Bahnen und Centren, Wiesbaden, 1904. 

2 Flechsig, Gehirn und Seele, ii. Aufl., Leipz., 1896, Taf. iv. Flechsig's views 
are presented in a condensed form in an article over his own signature in The 
Lancet, vol. ii. 1901, p. 1027. 

The references somewhat frequently made to Wilbrand and Saenger, unless 
otherwise indicated, refer to the volume above noted. Much use has been made of 
this valuable book, which is especially rich in its summary of the work of German 
writers and investigators. In order not to overload the chapter by a repetition of 
references, I might here call attention to some of the most important monographs 
or treatises used in its preparation, although I cannot make this list complete. 
These include Kussmaul's Disturbances of Speech in Ziemssen's Cyclopaedia of the 
Practice of Medicine, Amer. edition, vol. xiv., New York, 1877; Ferrier's Functions 
of the Brain, 1886, and Croonian Lectures on Cerebral Localization, 1890; Gowers's 
Diseases of the Nervous System, 1893, and his Clinical Lectures on Subjective Sen- 
sations of Sight and Sound, 1904; Wyllie's Disorders of Speech, 1894; and v. 
Monakow's Gehirnpathologie, Wien, 1897. Although it is giving scant justice to 
others, space will only permit me by naming them to acknowledge my great in- 
debtedness to Hitzig, Munk, Horsley, Schiifer, Beevor, Saenger Brown, Dejerine, 
Henschen, Pick, Bateman, de Schweinitz, Swanzey, Oliver, and Elder, among many 
others. Besides this general acknowledgment, references are made in various parts 
of the chapter to particular contributions. 



60 THE EYE AND NERVOUS SYSTEM 

having special relation to the evolution of particular functions. Some 
of the areas appear at the same time, others at a time separated by weeks 
or months from the areas which have preceded them. Thirty-six of 
these areas have been marked out by Elechsig on the cerebral cortex. 




Fig. 1.— Flechsig's primordial and association areas on the lateral aspect of the human hemicerebrum ; 

the primordial areas are shaded. 

In this connection our chief concern is with Flechsig's great sub- 
divisions of the cortex into: (1) primordial zones, and (2) association 
areas. The primordial zones correspond to the so-called projection 
areas, the regions of cortical representation of the special senses, com- 
mon sensibility and of movements. The association areas occupy a large 




Fig. 2.— Flechsig's primordial and association areas on the mesal aspect of the human hemicerebrum ; 

the primordial areas are shaded. 

portion of the cerebral surface, nearly two-thirds in all. They are situ- 
ated in those regions of the brain which were in times past often desig- 
nated as latent simply because we did not know their functions. Instead 
of being functionless they are the seat of the highest and most special- 



THE PSYCHOLOGY OF VISION 



61 



ized cerebral attributes. These association areas were divided by Flech- 
sig into two, a posterior and an anterior. The posterior association area 
occupies both the lateral and the mesal aspects of the brain and includes 
portions of the parietal, temporal and occipital lobes. To it Flechsig 
also gave the name of intermediary area. The anterior association area 
which is situated exclusively in the prefrontal region has also been 
designated by Flechsig as the terminal zone. These primordial and 
association areas are represented diagrammatically in Fig. 1 and Fig. 2. 




Fig. 



CONCEPT 



-Diagrammatic scheme of the physiological areas and centres of the lateral aspect of the human 

hemicerebrum. 



I have suggested that the term concrete concept area should be 
applied to Flechsig's posterior or intermediary area. It includes the 
optic memory field of Wilbrand and Saenger and much more, the mem- 
ory field directly related to all of the senses ; indeed, another designation 
which would be expressive of the functions of this great area would be 
the concrete memory field. This concrete concept area or concrete 
memory field is indicated in the diagrams, Figs. 3 and 4. This area 
is much subdivided according to the evolution of function. Tentatively 
its most important separate parts or centres might be indicated as repre- 
sented in the diagram, Fig. 3. The views of Campbell, based on histolog- 
ical research regarding the physiological subdivisions of the cortex, are 
given with illustrations, See Figs. 11 and 12, pages 73 and 74. These 
views, arrived at by a different road, are similar to those of Flechsig and 
the writer. 



62 



THE EYE AND XERVOUS SYSTEM 



Concrete concepts are of various kinds according to the degree of 
their simplicity or complexity. One may have a simple visual con- 
cept of a leaf or flower, made up purely of visual sensations and 
memories. This concept, however, becomes more complex, and at 
the same time more complete, when with it are associated the memo- 
rial images of the same flower or plant which are derived through 
other senses, — as those of touch and smell. This higher concrete con- 
cept can only be brought about by associations between visual and 
other sensory areas. Moreover, to the concrete concept, simple or 
complex, a name comes to be given through a series of cerebral pro- 
cesses which have their structural substrata in the higher auditory 




Fig. 4.— Diagrammatic scheme of the physiological areas and centres of the mesal aspect of the human 

hemicerebrum. 



sphere; and to connect these with the visual areas, other associating 
tracts must be present. The highest psychical area of the brain, that- 
concerned with attention and volition, with abstract concepts of every 
sort, is associated with the visual cortex, as it is with all other parts of 
the great concrete memory field ; for it is only by such association that 
reason, judgment, and fancy can be brought to bear in connection with 
our concrete ideas. 

A few words might be said here regarding the differences between 
the lower and the higher visual areas in the lower animals and in man, 
in order to emphasize the manner in which the secondary or higher 
visual centres develop as evolution proceeds. 



THE PSYCHOLOGY OF VISION 



63 



The lower or primary cortical visual area is largely developed in 
animals much below man which use their peripheral and central vision 
for some purposes even in a higher degree than man. They see acutely 
for long distances and have a large peripheral field. The optic memory 
field in animals below man and in savage races is highly developed as 
regards memories concerned with the recognition of those natural ob- 
jects, places and persons, an accurate knowledge of which is necessary 
for them for defense and subsistence. The visual portion of the concrete 
memory field in the highly civilized human being is greatly enlarged 
and much elaborated in accordance with his acquirements. 

The subdivisions given of the areas for concrete memories are of 




Fig. 5. — Scheme showing the extent and subdivisions of the secondary or higher visual cortex on the 
lateral aspect of the cerebral hemisphere. 

course provisional, and will doubtless be largely added to when our 
knowledge of the regional morphology, physiology and anatomy of the 
brain is increased. In Figs. 5 and 6 are shown the subdivisions of the 
visual areas on both the lateral and the mesoventral aspects of the hemi- 
sphere according to the writer's latest views. 

It will now be necessary to speak of the connections and associa- 
tions of the visual cortex. Between the calcarine and cnneal cortex and 
the pregeniculum and pulvinar of the thalamus runs a broad band of 
fibres long known as the optic radiations of Gratiolet, while the optic 
tracts connect the geniculate and thalamic visual centres with the retina. 
The fibres from the pulvinar run in the dorsal, those from the pregen- 
iculum in the ventral section of these bundles. It is not improbable 



G4 THE EYE AKD NERVOUS SYSTEM 

that in the visual radiations are fibres of at least two different origins, 
some fibres passing from the basal centres to the cortex and others from 
the cortex to these centres. 

We shall not here go into details of the exact manner in which these 
connections from the retina and the occipital cortex are made, except to 
say that it is after such fashion that similar halves of the retina, of both 
eyes, are related to the occipital cortex on one side ; the right halves of 
the retina to the right occipital cortex, and the left halves to the 
left occipital cortex ; while a small central macular field has a bundle of 
its own which also makes connection with the cortex of the brain on both 
sides. Another connection of the retina through the optic tracts is with 
the anterior colliculus of the quadrigeminum, this having a path to the 
occipital lobe and others to the nuclei of the ocular muscles. The reader 




Fig. 6.— Scheme showing the position of the primary visual centre on the mesal aspect of the cere- 
bral hemisphere ; and also the probable positions of the centres for color recognition, for elementary 
forms, and for those ocular movements which are immediately responsive to visual impressions. 

is referred to Dr. Spiller's article for a fuller consideration of the visual 
pathway. The entire retino-intracranial visual apparatus is well shown 
in the schematic diagram of von Monakow which is partly based on the 
work of Ramon y Cajal. The illustration is reproduced by permission 
of Professor von Monakow. I have modified it so as to introduce connec- 
tions of the primary optic centres with the higher visual field. It is 
based upon von Monakow's experimental and pathologico-anatomical 
investigations. 3 

3 Von Monakow, Arch. f. Psych., vol. xx. 3, and Gtehirnpathologie, 1897. 



THE PSYCHOLOGY OF VISION 65 

With the anatomy, physiology and diseases of the optic radiations, 
the basal optic centres, the optic tracts and the retina, I am not concerned 



MULLEB. S STTP- 
POBTING- CELLS 



OPTIC NERVE 




COLLECTING CELLS 



OOE.TIOAI. LATEE 



Fig. 7.— Scheme by von Monakow of the regions of the optic pathway (opticus) in the retina, and 
its further termination in the cortex of the occipital lobes. A, cones ; B, rods ; C, nuclei of the rods ; 
D, bipolar ganglion cells for the cones ; E, bipolar ganglion cells for the rods ; F, large multipolar 
ganglion cells (cells of origin of the optic nerve); G, centrifugal fibres of the optic tract and nerve 
which originate in the pregeminum and end in the retina ; H, reticular receiving cells of the pre- 
geniculum: and I, the optic radiation cells of the pregeniculum. The course of the light waves 
towards the cortex is indicated by way of cones and rods ; bipolar ganglion cells for the cones and 
rods (optic cells); receiving cells in the pregeniculum, optic radiation cells; cortical cells (third and 
fifth layer). In order to complete the cortical connections of the optic pathway an addition has 
been made to the diagram at H V, which indicates the higher visual cortex in the angulo-occipital 
region which is connected with the lower visual cortex by the lines running in the direction of the 
arrows. It must be remembered in studying this diagram that only the connections to one side of 
the occipital cortex are given. The semidecussation of the optic tracts is not represented. In addi- 
tion, it must be recalled that tracts run from the primary visual cortex of each occipital lobe to the 
higher visual cortex of the opposite side. This also is not represented in the diagram, which is 
intended simply to give a clear conception of the connections of the retina with the basal ganglia and 
the primary visual cortex (primary optic centres). 



in the present chapter. It is only necessary to recall here the fact that 
hemianopsia, of the most complete type, has its pathological seat in a 
lesion which severs the optic radiations or optic tract. 
5 



66 THE EYE AND NEKVOUS SYSTEM 

Special sets of fibres associate the primary visual centres or optic 
perception fields of both hemicerebrums with the higher cortical centres 
of each side, as will be further discussed when the two clinical forms of 
word-blindness are considered. It is important that this double associa- 
tion should be borne in mind. This association is indicated diagrammat- 
ically in Eig. 18, in which O 1 and O 2 represent respectively the left 
and the right occipital lobe. According to Dejerine, it is brought about 
by the fibres from the cortex of the calcarine fissure of one side crossing 
through the forceps major in the splenium of the callosum, and joining 
with the fibres from the calcarine cortex of the other side, the united 
bundle then passing to the higher visual cortex. 

A matter of considerable interest is that of the existence or non- 
existence in the carrefour sensitif of Charcot, or in juxtaposition to it, 
of a tract concerned with the transmission of visual impressions. Some 
of the older records of cases would seem to bear out the theory of the 
existence of such visual fibres in this position, but the weight of opinion 
is against this view. Most, if not all, of the cases are more readily ex- 
plained on the theory of implication of the nearby optic radiations in 
the lesion which impairs or destroys the posterior bundle in the posterior 
limb of the internal capsule. 

Besides the tracts which associate the visual perception centres of 
both hemispheres with the visual memory field of each side, other asso- 
ciating tracts must be taken into consideration in discussing the func- 
tions and focal diseases of the visual cortex. These associations are, in 
fact, so numerous that the subject would become befogged, at least for 
the purposes of the physician, if an attempt was made to discuss them in 
great detail. In the first place, the various subdivisions of the higher 
cortical visual field have more or less intimate associations with each 
other, in accordance with the degree of association between their func- 
tions. The centres in the sphere of object seeing which are concerned 
with the recognition of persons, places and natural objects are associated 
with the centres of language symbolization. The centres of the higher 
visual area generally are connected by associating tracts with similar 
concept centres in other portions of the concrete memory field, as, for 
instance, with the higher centres of hearing, touch, taste and smell. 

HISTOLOGY OF THE VISUAL CORTEX, ESPECIALLY 
WITH REFERENCE TO VISUAL LOCALIZATION. 

As in other parts of the cerebral cortex, histology throws some light 
upon localization. What is known as the layer of Gennari occupies a 
position which is supposed to indicate the extent and limitation of the 



HISTOLOGY OF THE VISUAL COETEX 67 

primary visual cortex in man and the lower animals. The layer of 
Gennari spreads over the cortex in such a way as to include the lingual 
lobule from the junction of the calcarine and parieto-occipital fissures, 
caudad to the occipital pole, including the calcarine fissure, the entire 
cuneus, and the first occipital convolution. Fibres from the optic 
radiations have been traced to all the regions in which this layer is 
situated. 

Space cannot be given to a full consideration of the minute anatomy 
of the visual cortex and its bearings upon the question of the develop- 
ment and the localization of cortical visual centres. 

Meynert believed that in the occipital lobe the classic arrangement 
of the cortex into five layers, as especially seen in the motor region, was 
not followed. He recognized no less than eight layers. The present 
tendency is, following Ramon y Cajal, to recognize the same number of 
layers in the visual as in other portions of the cortex. Counting from 
without inwards, Cajal described five layers, — namely, (1) a molecular 
layer ; (2) a layer of small fusiform vertical cells ; (3) a layer of small 
pyramidal cells ; (4) a layer of large pyramidal cells ; and (5) a layer 
of polymorphous cells (Fig. 8). An acceptation of the views of Cajal 
brings the occipital lobe in harmony with other portions of the cortex, 
with primary functions like hearing, touch, smell, taste and the repre- 
sentation of movement. The studies of Cajal were made upon small 
mammals. The same layers modified by evolution are, however, recog- 
nizable in man and in the primates below man. 

In Fig. 9 is shown a crossed section through the normal cortex of 
the left calcarine fissure in an eleven-year-old epileptic studied by 
von Monakow. The right hemisphere was the seat of a porencephalic 
defect. 

Von Monakow's method of considering the visual cortex is exhibited 
in this and in other illustrations in his work. Regarding the subject in 
a purely topographical sense, the following layers according to him are 
recognizable in the cortex of the occipital lobes : (1) the ependymal and 
fusiform layer similar to the layers found elsewhere in the convolutions ; 
(2) the layer of the small pyramidal cells, although even here there are 
a few examples of the large pyramidal cells; (3) the layer of giant 
pyramidal cells between, below, and above which are found accumu- 
lations of nuclei ; (below the layer of the small pyramidal cells is 
the upper portion of the Vicq d'Azyr stripe and below this a collec- 
tion of substantia gelatinosa — the lower part of the Vicq d'Azyr stripe 
extends into the fourth layer) ; (4) the fourth layer is constructed 
chiefly of polymorphous multipolar cells and contains many myelinated 



68 



THE EYE AND NEKVOUS SYSTEM 



fibres ; it is rich in ground substance ; ( 5 ) this layer is constructed of 
elements similar to those of the fourth layer, but it is indicated as a sep- 



n 



in 



i&W- 



IV 



VI 



EV 



pPy 



gPy 



Pm 




4. 



Fig. 8.— Scheme representing the layers of the 
occipital cortex according to Cajal ; I, II, in, iv, 
V, are the five layers of Cajal ; M, the molecular 
layer ; Fv, the layer of fusiform vertical cellules ; 
pPy, layer of the small pyramidal cells ; gPy, layer 
of large pyramidal cells ; and Pm, layer of poly- 
morphous cells. 



Fig. 9.— Crossed section through the normal 
cortex of the left calcarine fissure in an eleven- 
year-old epileptic with porencephalic defect in 
the right parietooccipital lohes, and total primary 
destruction of the optic radiations on the right 
side, magnification 300. The five layers of the cor- 
tex are after Meynert, — K, nuclei ; P, giant pyrami- 
dal cells; m, subcortical white matter, (von 
Monakow.) 

arate layer because of the less number of horizontal nerve fibres which 
it contains. 



HISTOLOGY OF THE VISUAL COETEX 69 

A comparative histological study of the cortex of animals having 
vision, from the lowest to the highest, and a similar comparative study 
of the lower and higher visual cortical areas in man, hear out the views 
as to function hased on other methods of investigation, as those of physi- 
ology and clinical pathology. Much work, extending over many years, 
has been done in this field of research, but the most valuable is without 
doubt that recently published by F. W. Mott and A. W. Campbell. The 
correlation of structure and function in the neuro-visual apparatus was 
demonstrated by Mott, 4 as exhibited in changing cell lamination of the 
visual cortex of mammalia from insects to the primates. The transition 
from monocular to binocular and from panoramic to stereoscopic vision 
is discussed, and the relation of such transition to full decussation and 
semi-decussation of the optic tracts is shown. The semi-decussation is 
correlated with "progressive development in the layer of the higher asso- 
ciational pyramidal cells lying above the layer of the granules in the 
cortex". 

Mott illustrated the various stages of evolution of the visual cortex 
by actual preparations. He begins with a discussion of the extremely 
simple type of visual cortex shown by the mole and the shrew, with their 
limited visual powers. In these animals the cell structure of the cortex 
is of the most rudimentary character, consisting of small stellate or 
quadrilateral cells resembling granules, below which is a thin layer of 
polymorphic cells with a few large cells. These three types of cells, 
rudimentary in the mole and the shrew, are found throughout the series 
of animals studied by Mott. In rodents which have wide panoramic 
vision, but unimportant elaborations and associations, the visual cortex 
becomes considerably more complex. 

In the rabbit is a higher elaboration of the visual cortex proportion- 
ate to the increased visual powers. Here are seen the visuo-sensory cells 
of Kaymon y Cajal, a thin layer of large stellate cells, and closely packed 
pyramidal cells just above the granules. Up to this point in Mott's 
series the vision of the animal studied is chiefly panoramic or periscopic, 
being characterized but little by comprehension of depth and special 
form. Mott traces his series through the marsupials, ungulates, the 
canines and the felidse, each mammalian form in the advancing series 



4 Mott, The Lancet, vol. ii., December 3, 1904, p. 1555. Mott's results were 
presented in the Bowman Lecture, and are recorded in an authorized and cave- 
fully prepared abstract in the Lancet, from which the account here given is 
taken. I have reproduced, by permission of Dr. Mott, the illustrations given in Fig. 
10, which represent two histological types of the visual cortex, — namely, in the 
mule-deer and macacus-monkey. 



/ 



70 



THE EYE AND NEKVOUS SYSTEM 




in 



IV 



Ilia 



III b 



IIIc 



IV 




II 



in 



IV 




A— Mule-Deee. 



B— Monkey. 



C— Monkey. 



Fig. 10.— Reproduction of drawings after photomicrographs of sections of the visual cortex in the 
mule-deer, and of the visuo-sensory and visuo-psychic cortex in the monkey (macacus). Magnification 
150. The percentage of thickness of the different layers is only approximate. 

A, Mule-deer, the visuo-sensory area : I, 20 per cent.; II, 20 per cent.; scattered pyramidal and large 
stellate cells ; III, 11 per cent, granules ; IV, 48 per cent, solitary cells of Meynert ; polymorphic. 

B, Monkey, the visuo-sensory area: I, 10 per cent.; II, 27 per cent, pyramidal; III a, 10 per cent, 
outer layer of granules ; III b, 10 per cent, large stellate cells, line of Gennari ; III c, 17 per cent, inner 
layer of granules ; IV, 11 per cent, inner line of Baillarger, with solitary cell of Meynert ; V, 15 per 
cent, polymorphic. 

C, Monkey, the visuo-psychic area : I, 17 per cent.; II, 43 per cent, pyramidal ; large pyramids ; III, 
14 per cent, granules ; IV, 10 per cent, inner line of Baillarger, with solitary cell of Meynert ; V, 16 per 
cent, polymorphic. (Mott.) 



HISTOLOGY OF THE VISUAL COETEX 71 

showing greater extent and elaboration of the visual cortex in accordance 
with the addition of new powers of vision, binocular and stereoscopic 
perception being added to uniocular and panoramic vision. The cat is 
probably the first animal in the series presented by !AIott which is capable 
of convergent stereoscopic vision by movement of the eyes independent 
of the head. Below the cat complete decussation of the optic tracts is 
probably present. In this animal an imperfect semi-decussation, like 
that which is later seen in its fuller development in the primates and 
man, takes place. The cat is probably the first species in the series of 
mammals in which section of the optic tract produces hemianopsia. The 
visual cortex is developed proportionately in each species according to 
its increased powers. In the felidse, to which the cat belongs, the pyra- 
midal area is of increased depth, the solitary cells of Meynert very 
numerous, and the polymorphic layer diminished as compared with 
series lower in the scale. 

Reaching the primates with their perfect binocular stereoscopic 
vision, which can be added to by movements of the eyes and head, a 
semi-decussation of the optic tracts, a distinct macula lutea, and a defi- 
nite occipital lobe with the line of Gennari visible to the naked eye, are 
found. For the first time the visual cortex presents two structurally 
different regions, a visuo-sensory and a visuo-psychic. The visuo-sen- 
sory cortex attains its maximum extent and complexity in the ape and is 
characterized by increase of width of the line of Gennari, a double layer 
of granules, and above the outer of these a double layer of small and 
medium-sized pyramids. The visuo-psychic or associational cortex (not 
definite as a distinct zone below the ape) is characterized by an increased 
depth of the pyramidal layer owing to the presence of a third row of 
large associational pyramids, this region being progressive in extent 
upwards to man, in whom it attains its maximum development at a 
period subsequent to birth. The visuo-sensory and visuo-psychic cortical 
areas correspond to the primary and to the higher cortical visual areas 
respectively. The results of the researches of Campbell, which will next 
be referred to, are practically the same as those reached J)y Mott, al- 
though the investigations were in the main along different lines. 

Campbell 5 has pursued a series of investigations on the medulla ted 
structure of the nerve fibres of the cerebral cortex, and also of the cor- 
tical cells, with the result that these investigations have served to show 
that such studies constitute a method of determining the functions of 



5 Campbell, Proceedings of the Royal Society, vol. Ixxii. Dec. 3, 1903; Journal of 
Mental Science, October, 1904 



72 THE EYE AXD XEKVOUS SYSTEM 

different regions of the cerebral surface as valuable as physiological 
investigations on the lower animals, embryological researches like those 
of Elechsig on the fetal brain, clinicopathological records of well ob- 
served cases, and morphological and anatomical studies of fissural and 
gyral development and arrangement. He has shown that histological 
studies of the form and arrangement of the nerve elements in the normal 
adult, the microscopical change present in pathological cases, and the 
relative histological findings in anthropoid apes and in man, all tend to 
one end — the demonstration and verification of the existence of specially 
arranged separate functional areas and centres. Among the clinico- 
pathological cases those examined were instances of amyotrophic lateral 
sclerosis, of amputation of extremities, and of deprivation of the special 
senses. The results obtained are of the greatest interest, and in the main 
are confirmative of the views of Flechsig, of the morphological and ana- 
tomical studies of E. A. Spitzka and the writer, and of the clinicopatho- 
logical facts accumulated during recent years by a large number of con- 
tributors to neurology. 

" Viewed collectively/' Campbell says, " the human brain harbors 
two varieties of centres, controlling what we may call ' primary' and 
' higher evolutionary' functions respectively ; the former are those com- 
mon to all animals and essential to survival, — viz., centres for movement 
and common and special sensation ; the latter are those complex psychic 
functions in the possession of which man rises superior to all other 
beings." 

Among Campbell's conclusions are (1) the motor region is ceph- 
alad of the central fissure; (2) an area for higher skilled move- 
ments, which Campbell calls the " intermediary precentral" area, just 
in advance of the motor area, corresponds practically to the so-called 
motor centres for speech and writing; (3) the prefrontal lobe, rela- 
tively weak in nerve cells and fibres, is the last pallium of the human 
brain, its functions as yet being uncertain; (4) the post-central con- 
volution is the main terminus for common sensory impressions; (5) 
the post-central gyre is probably a primary area, one serving for the 
recognition of the simplest components in common sensation, as impres- 
sions of heat and pain; (6) just behind the post-central area is an 
intermediate post-sensory area concerned with the elaborations of sensa- 
tions like muscular sensibility and stereognostic perception. 

With regard to the visual cortex, with which I am here particularly 
concerned, the views of Campbell are in keeping with what has been 
indicated as his opinions regarding other portions of the cerebral sur- 
face ; moreover, these views are in accord with those of Elechsig, the 



HISTOLOGY OF THE VISUAL COKTEX 



73 



writer and others, who have endeavored to show that a large part of the 
parieto-temporo-occipital cortex should be regarded as a great concrete 
memory field, a region higher than those concerned with the mere recep- 
tion of sensory impressions and the projection of impulses concerned 
with movements. Campbell believes, as the result of his studies, that 
the visual area is histologically divisible into two areas. The first of 
these, according to him, closely follows the calcarine fissure, extending 
only a short distance from it on each side. It is, he believes, intended 
for the reception of primary visual impulses. To it he gives the name 
visuo-sensory area. The second area skirts and invests the first, and is 
termed visuo-psychic, its purpose being the further elaboration of visual 

Precentral Postcentral Intermediate postcentral 



Intermediate precentral 



Parietal 



Frontal • . 



Visuo-psychic 




Prefrontal 



Common temporal 
"Audito-sensory 



Audito-psychic 

Fig. 11.— Scheme of the lateral aspect of the left hemicerebrum showing the position of the primary 
intermediate cortical functional areas. The primary or lower areas in the case of the special senses are 
designated, respectively, audito-sensory and audito-psychic, and visuo-sensory and visuo-psychic. The 
visuo-sensory area is present over a very limited field about the occipital pole, while the visuo-psychic 
spreads over a much larger area of the lateral occipital lobe. (A. W. Campbell.) 

sensory impressions. It is characterized by a remarkable wealth of 
nerve fibres, coupled with the presence of curious large pyramidal cells. 
The various affections included under the general designation of psychic 
blindness are to be attributed to the destruction of this visuo-psychic 
area. 

A study of the diagrams of Campbell (Figs. 11 and 12) shows that 
his visuo-sensory and visuo-psychic areas correspond in a general way 
to the subdivision of the visual cortex as given by me in this article. 
They differ somewhat, however, as regards the limitations and exten- 
sions of each of the areas. In accord with Henschen, he relates his 
primary or visuo-sensory area quite closely to the calcarine fissure. I 



74 



THE EYE AND NERVOUS SYSTEM 



believe it should be extended so as to include the whole of the cuneus, 
and possibly a narrow fringe of cortex on the lateral aspect of the occip- 
ital lobe. He restricts his visuo-psychic area to the occipital convolu- 
tions both on the mesotentorial and lateral aspects of the hemisphere, 
while I would extend it forward on the latter, so as to include the caudal 
extremities of the parietal and temporal convolutions. The principle, 
however, is the same. 

In a general way the histological investigations of Campbell show 
that the posterior association area of Flechsig, the concrete concept area 



Postcentral 
Intermediate postcentral 



Precentral 



Intermediate precentral 



Limbic 
Variations at B and C 



Parietal 



Frontal 



Prefrontal 



Visuo-psychic 



Visuo-sensory 




Visuo-psychic 



Common temporai 



Pyriform 



Fig. 12.— Scheme of the mesal and mesotentorial aspect of the left hemicerebrum. The visuo-sen- 
sory area as here indicated follows closely the calcarine fissure, while the visuo-psychic extends beyond 
the calcarine cortex in both directions. The various cortical subdivisions are indicated with clearness 
both graphically and descriptively. (A. W. Campbell.) 



of the writer, is subdivided into sub-areas for the representation of con- 
crete memories, those memories which are derived primarily by way of 
common sensibility and special sensation ; they are, in other words, 
higher areas of vision, audition, common sensibility, olfaction and gus- 
tation. 

One point stands out prominently in Mott's investigations, — namely, 
that as vision advances increase in the development of the pyramidal 
cells takes place. Both in the race and in the individual development 
of the pyramidal cells, in number and in size, increases step by step 
with the evolution of power. Mott refers to the well-known myelination 
views of Flechsig, especially with reference to the optic radiations and 
the development of the different lavers of the visual cortex. The inter- 



CORTICAL VISUAL AREAS 75 

mediate zones of Campbell are but other names for the association areas 
of Mechsig or for the concrete concept or concrete memory field of the 
writer. In the visual cortex the intermediate visuo-psychic area is the 
optic memory field of Wilbrand and Saenger, the region already dis- 
cussed as the seat of those higher visual powers such as the recognition 
of persons, places, objects, designs, and the symbols of language. 

In his discussion of the development, structure, and function of the 
visual cortex, Mott refers to the morphological and anatomical studies 
on the brains of individuals of low and high development, and of those 
of different races, as of the Soudanese and Fellaheen, also of the Chinese, 
Goanese, Congo negro and the Egyptian. The work of E. A. Spitzka 
on the brains of men distinguished for their intellectual powers, and that 
of the writer on the brains of paranoiacs, imbeciles, negroes and crim- 
inals bear out the views of Mott. 

POSITION AND THE SUBDIVISIONS OF THE 
PRIMARY CORTICAL VISUAL AREA. 

The subdivision of the visual cortex into a primary or lower and a 
secondary or higher visual region having been considered, and such sub- 
division having been seen to be in accord with the views of Elechsig and 
the similar, although otherwise expressed, views of Mott and Campbell 
as to primordial and association areas, in the further discussion of our 
subject it is next necessary to glance at the question of the exact position, 
and the extent and subdivision of the primary cortical visual area. 
While all authorities are in practical accord in placing the primary or 
lower visual area, in whole or in very large part, on the mesotentorial 
surface of the hemicerebrum, they differ considerably with regard to 
the exact limitations and extensions of this physiological region. 6 

In studying the literature of the subject one is first struck with the 
great differences between authorities with regard to the extent of the 
primary cortical area. The extreme on one side is represented by the 
view of Henschen," that the entire optic perception field is confined to 



s Dr. Wm. G. Spiller. in his chapter in this work on the Intracranial Portion 
of the Encephalic Xerves. Especially of Those Related to the Eve. in the section 
on Primary Optic Centres, goes at some length into the question of their position, 
and the reader is referred to this portion of his article in connection with what 
is here said. It is necessary, however, to completeness, and in order that the exact 
views of the writer with regard to cortical physiology of vision may be understood, 
that he should here give some consideration to the location and extent of the 
primary cortical centres for sight. 

7 Henschen. Klin. u. anat. Beitr. z. Pathol, des Gehirns. Upsala. 1S90-1903. 



76 THE EYE AND NEKVOUS SYSTEM 

the calcarine fissure, and the hypothesis is based on the fact that in one 
of his own cases of hemianopsia the lesion was really or apparently 
restricted to this fissure ; and on the other hand by the view of Vialet, s 
that the primary visual area of the cortex includes the entire mesoten- 
torial surface of the occipital lobe. Seguin 9 confined the primary cen- 
tres for vision to the cuneus. Not a few of the physiologists and clinico- 
pathologists who have written on the subject have included not only the 
entire mesotentorial occipital cortex, but also a large part of the lateral 
occipital lobe, and some like Ferrier have even extended the region 
into the temporal and parietal lobes. It must be borne in mind, how- 
ever, that some of these writers have not made the distinction which is 
drawn here and should always be made between the primary and the 
secondary visual regions of the cortex. Not a little confusion has arisen, 
especially in the earlier consideration of the subject, by a non-recogni- 
tion of this distinction. The only truly primary or primitive visual 
function is the recognition of luminosity, or perhaps it would be better 
to say the recognition of lightness or darkness. Color recognition, 
and that of form, come later in evolution. In a strict sense, therefore, 
the most primitive centres for vision are those concerned only with the 
appreciation of lightness and darkness. I believe that the centres for 
luminosity, color and form are distinct in the cerebral cortex. The 
color centres and the centres for form have developed out of the centres 
for light, just as the ability to recognize color and form has developed 
upon the basis of luminous recognition. This subject will be more fully 
treated when the cortical centres for color are discussed. It will only 
be necessary for clearness to state here, that it is view of the writer 
that the centres for color are contiguous to the primitive centres for 
sight, and that it is probable that they are located in the lingual and 
fusiform lobules below the calcarine fissure, while the centres for form, 
beginning at the mesal edge of the occipital portion of the hemisphere, 
are spread out in more and more complex development on the lateral 
aspect of the hemicerebrum chiefly in the occipital convexity. The pri- 
mary centre for vision as here defined is, I believe, situated in the 
cuneus, the calcarine fissure and the cuneal slope of the parietooccipital 
fissure. 

This primitive cortical area for sight, located in the cuneus and the 
calcarine and parieto-occipital fissures, has in the first place, two sub- 
divisions, a peripheral and a central. The central subdivision is for the 
cortical representation of the macula and the peripheral for the rest of 

"Vialet, Les centres cerebraux de la vision, etc., Paris, 1893. 
8 Seguin, Jour. Nerv. and Ment. Dis., vol. xiv., 1887. 



COKTICAL VISUAL AEEAS 77 

the retina. In the affection known as hemianopsia frequently the blot- 
ting out of the visual field is not, strictly speaking, as the name indi- 
cates, for exactly half of this field but for one-half of it less a small 
semicircular area which is inserted notch-like into the hemianopsic area 
at its internal central part. As Swanzey 10 expresses it this small area 
of retained vision is bitten out of the blind field. A patient may have 
double hemianopsia as the result of successive attacks involving both 
cerebral hemispheres in the occipital region, and circumscribed central 
vision remain. 

These and other facts would seem clearly to point to separate regions 
of representation for the central and for peripheral portions of the 
retinal field respectively. As will more fully appear later, I hold that 
both in the lower and in the higher visual fields, the macula and the 
peripheral retina are separately represented, but first let me speak of 
the probable position of the separate centres for the macula and periph- 
ery in the primary cortical visual area. For the present I believe it 
is best to accept the view of Henschen which has his own well recorded 
case in its support, and a few other facts, that the centre for the macula 
in the primary visual cortex is situated in the more anterior portion of 
the calcarine fissure, that is towards the apex of the cuneus. 

Tentatively it may also be assumed that a horizontal zonular strip 
of the retina is represented in the posterior part of the calcarine fissure. 
These two localizations are based upon cases of hemianopsia with lesion 
of the middle portion of the calcarine fissure and adjoining cuneus, 
while the frontal and posterior parts have escaped. 

Cases of cortical disease, quadrant and sector hemianopsia, and cases 
with ring-like or other scotomata in the visual field, are on record. Such 
cases are only to be explained on the supposition of a subdivision of 
retinal representation in the visual half centres. Much difference of 
opinion has arisen as to how this subdivision takes place, and the cases 
reported in proof of various positions are somewhat confusing and con- 
tradictory. A case of Henschen, for example, would seem to show that 
the upper part of the retina is represented in the superior lip of the cal- 
carine fissure and the lower part in the inferior lip, and that a horizontal 
strip between the upper and lower parts of the retina is represented at 
the posterior extremity of this fissure ; but the weight of facts is cer- 
tainly against the view that the entire half vision centre is situated in 
the depth of the calcarine fissure. If this were true, in a case recorded 

10 Swanzey, Eye Disease and Eye Symptoms in Their Relation to Organic Dis- 
eases of the Brain and Spinal Cord, in System of Diseases of the Eye. edited by 
Norris and Oliver, Philadelphia. 1900. 



78 THE EYE ANY) XERVOUS SYSTEM 

by Beevor and Collier, 11 left hemianopsia should have been present, or 
at least left hemianopsia with the exception of a small area around the 
fixation point. The calcarine fissure, the inferior portion of the cuneus 
dorsal to it and the lingual lobule on its ventral side, were all destroyed, 
yet quadrantic hemianopsia alone, the fixation point escaping, was pres- 
ent. The only explanations which suggest themselves for Henschen's 
case are that in some persons retinal half representation is more concen- 
trated cortically than in others, or as suggested by Beevor and Collier, 
the hemianopsia was due to involvement of the optic radiations in addi- 
tion to the calcarine cortex. The case of Beevor and Collier is referred 
to in some detail by Spiller, see page 9, and will again be recalled 
when I consider cortical anopsias and hemianopsias. I shall only say 
here that it seemed to demonstrate that the lower quadrant of the visual 
field is represented by the upper or dorsal portion of the cuneus, as in 
their case the patient presented blindness of the left upper quadrant of 
the visual field, the fixation point escaping. 

The case of Hun 12 has long held an important place with regard to 
this question of the subdivision of retinal representation. In this case 
the defect of vision was in the lower left quadrant of each eye, and the 
lesion as recorded by Hun was atrophy of the lower half of the right 
cuneus. Hun argued from this case that the representation of the lower 
quadrant of the visual field was in the lower half of the cuneus. I have 
re-examined Hun's original report of this case, and am inclined to agree 
with Beevor and Collier and others in the view that the case does not 
fully support the contention that the lower half of the cuneus is the 
area of representation for the lower quadrants of the retina of the same 
side. It is notable in the record of the case that the necropsy was made 
thirteen days after death and after the body had been embalmed, and 
also that Hun reports that the subcortical white matter was involved in 
the lesion to the depth of at least one- third of an inch. Probably as no 
microscopical examination was made, and as a brain lesion usually ex- 
tends beyond the point where it can be traced by the naked eye, the 
degree of involvement of the subcortex and therefore of the optic radia- 
tions, was greater even than would be understood by the statement that 
the destruction extended to the depth of one-third of an inch. The case 
therefore has less value in the study of the subdivisions of retinal half 
representation than that of Beevor and Collier. In their case the lesion 
was studied with extreme care both macroscopically and microscopi- 
cally, and showed that the cortex alone was involved. 

11 Beevor and Collier, Brain, part 2. 1904, p. 153. 

12 Hun, Amer. Jour, of the Med. Sci., vol. xciii. Jan.-April, 1887, p. 140. 



CORTICAL CENTRES 79 

My conclusions with regard to the subdivisions of retinal representa- 
tion in the primary cortical visual centres are : ( 1 ) that a lower quad- 
rant of the field, or a little less than a quadrant, is represented in the 
upper half or two-thirds of the cuneus ; (2) that the upper quadrant 
or a little less than a quadrant, is represented in the lower half of the 
cuneus, including the middle portion of the calcarine fissure; (3) that 
the macula, or at least the fovea, is represented in the anterior ex- 
tremity of the calcarine fissure and its cuneal border; and (4) that a 
zone or strip of the peripheral retina is represented in the posterior 
extremity of the calcarine fissure and its cuneal border. When I 
speak of a little less than a quadrant being represented, it is because the 
strip supposed to be represented in the posterior limits of the cal- 
carine fissure passes horizontally from about the line of the fixation 
point through the peripheral retinal field. It will be seen that in com- 
ing to these conclusions I have adopted in part the views of Henschen 
and in part those of Beevor and Collier. Doubtless the differentiation 
of retinal representation in the primary visual cortex is much greater 
than is here indicated, but the facts at command only permit at present 
this degree of subdivision. 

FOCAL DISEASES OF THE VISUAL CORTEX. 

The focal diseases of the visual cortex are those disorders of sight 
and intelligence which result from lesion or disturbance of any part of 
the lower or higher visual zones. The most important of these affec- 
tions and those which will be considered in the present chapter are: 
(1) cortical anopsias and hemianopsias; (2) hemiachromatopsia and 
other forms of color-blindness of cortical origin ; ( 3 ) central ambly- 
opias crossed and uncrossed; (4) mind-blindness, total or partial, and 
transient or permanent ; ( 5 ) word-blindness, letter-blindness and num- 
ber-blindness ; and (6) optic aphasia. Various disorders dependent 
upon visual cortical disease or related to it, such as alexia, paralexia, 
dyslexia, visual agraphia, paragraphia, and dysgraphia, will receive 
consideration, as will also the cortical and transcortical mechanism of 
visual spectra and aura\ 

HEMIANOPSIAS AND OTHER DEFECTS IN THE VISUAL 
FIELDS DUE TO CORTICAL LESION. 

It will not be necessary to go at length into a consideration of 
the first of the classes of focal disease due to lesions of the visual cortex, 
the hemianopsias and the other more restricted defects. The subject 



80 THE EYE AND NERVOUS SYSTEM 

lias already necessarily received some consideration when discussing the 
position, limitations and subdivisions of the primary cortical visual 
area, and it has also claimed the attention of Dr. Spiller in his discus- 
sion of the primary optic centres. The unusually interesting cases of 
Hun and of Beevor and Collier have been given, and some reference 
has been made to other cases. 

A word or two concerning the terms most commonly used in de- 
scribing the diseases of the primary cortical areas may serve to keep 
the subject well in hand. In the first place it should be remembered 
that hemianopsia, of whatever type, may be partial or complete. It 
may be partial as regards the amount of the visual field obscured or 
with reference to the degree of this obscuration. An absolutely total 
hemianopsia is one in which the dividing line between the blind and 
the unimpaired field passes through the fixation point. In practice, 
however, it is customary to describe those cases as hemianopsic in 
which there is a small central area of retained vision, a fact which has 
been referred to several times. In quadrant anopsia only one-half of 
the half-field is obscured. A few almost typical cases of this form 
of impairment of the visual field have been recorded. In some cases, 
however, a little less than a quadrant of the entire visual field is lost, 
a ribbon-like area of retained vision being left at about the position 
of the horizontal meridian. In double hemianopsia, as the result of 
simultaneous or successive attacks of homonymous lateral hemianopsia, 
the entire field of vision usually, with the exception of a small central 
area, is obscured. By a relative anopsia or hemianopsia is meant a 
partial impairment of the power of visual recognition in a portion or 
in one-half of the visual field. This relative hemianopsia may take 
several forms. The patient may, for example, in the impaired field 
recognize the color, form, and luminosity of an object, but not with 
the same clearness and intensity as in the unimpaired field. Hemia- 
chromatopsia is, in a sense, a relative hemianopsia ; the patient, while 
not recognizing colors in the affected field, is able to recognize form 
and light. (See Chap, iv, p. 167.) 

Everyone familiar with the gross and microscopic pathological anat- 
omy of the brain knows that it is extremely rare to find disease 
strictly limited to the cortical cinerea. Hemorrhagic lesions almost in- 
variably involve both cortex and subcortex, and often the inner mem- 
branes as well. The same remark applies with increased force to ab- 
scesses of the brain; and tumors are almost never limited to the cor- 
tex, although growing, as they frequently do, from the membranes, 
they may for a time simply erode or displace it, later causing de- 






COETICAL CENTRES 81 

struction of both cortical and subcortical substance. Softening due to 
either embolic or thrombotic occlusion, while it often includes a large 
cortico-subcortical area, may be limited to either the subcortex or the 
cortex. What closure of a vessel will cause is, of course, conditioned 
by the parts supplied by it. In rare cases a cerebral hemorrhage in- 
volves so little of the subcortex as to make it practically a cortical lesion. 
From the above statements it will be seen that the classes of cases which 
throw most light upon the exact functions of cortical visual areas, as 
of other regions of the brain surface, are tumors growing from the 
membranes before they have invaded deeply the cerebral substance, and 
a few cases of necrosis restricted to the cortex. The cases of Hun and 
of Beevor and Collier were of the latter class. 

Cases of purely cortical or of nearly purely cortical hemianopsias 
or anopsias are extremely rare. A few have been recorded and are to 
be found in the most recent works on ophthalmology and neurology. 
Wilbrand and Saenger note the most important of these. In one case 
left-sided hemianopsia was present, a small portion of the visual field 
just beyond the fovea, three to six degrees, not being obscured. Xe- 
cropsy and microscopical examination showed an exclusively cortical 
softening in the depths of the calcarine fissure. (Holm.) In another 
case of complete right-sided homonymous hemianopsia, the necropsy 
revealed calcarine softening. (Hilden.) Another case of permanent 
hemianopsia with insular formed scotomata in the neighborhood of 
the horizontal meridian in which a cortical focus of softening was 
found in the floor of the calcarine fissure has been recorded. (Eggers.) 
In addition to these cases of what appears to have been strictly cortical 
lesion, a few others have been reported in which the disease extended 
very slightly into the subcortex. 

It sometimes happens that a lesion of the cortex of the lateral 
occipital lobe apparently causes a true hemianopsia, but in these cases 
the local pathological etiology is only apparent. A closer examination 
always shows that the lesion involves the optic radiations somewhere 
in their course from the pregeniculum to the calcarine fissure and 
cuneus. This is the explanation of a number of cases of hemianopsia 
which have been recorded as occurring in connection with lesions of 
the angular gyre and other parts of the lateral temporo-occipital region. 
Von Monakow gives an illustration of a case of this kind, the surface 
appearance being shown in Eig. 13. In this case, by making frontal 
sections through and cephalad of the lesion so as to expose the cortex, 
corona radiata and other related parts, it was found that the original 
lesion involved to some extent the optic radiations, in which also sec- 
6 



82 



THE EYE AXD XEBVOTTS SYSTEM 



ondary degeneration had occurred. Von Monakow gives illustrations 
of the hemianopsic fields. 

Double hemianopsia is of comparatively infrequent occurrence, but 
a sufficient number of cases have been recorded, both with and without 
necropsy, to shed some light upon its pathology in different cases. In 
most of the instances of this affection with necropsy both cortex and 
subcortex have been implicated in the lesion. It is indeed the rule 
in any case of hemianopsia when the cortex is involved for the sub- 
cortex also to take part in the disease, but the reverse is not so com- 
mon. In other words, lesions of the optic radiations, the cortex remain- 
ing free, are relatively frequent and of course, in those cases in 







Fig. 13.— Lateral aspect of the left cerebral hemisphere in a case of apparent cortical hemianopsia. 
H, lesion in the interparietal fissure, and also of the superior parietal convolution (P, i), which is in 
large part destroyed. The line 1-1 represents a transsection made through the hemisphere and the 
posterior portion of the lesion. The line 2-2 indicates the position at which a transsection was made a 
short distance in front of the lesion. It is so placed as to about pass through the angular gyre. These 
sections were made in order to study the relation of the lesion to the cortex and subcortex. The optic 
radiations were involved in the lesion in which also secondary degeneration had occurred. (Von 
Monakow. ) 



which the hemianopsia is due to lesion of the optic nerves, tracts, or 
pregeniculse, the question of cortical implication does not arise. 

In discussing double hemianopsias the fact already alluded to 
must be emphasized, that in lateral homonymous hemianopsia the loss 
sometimes extends over the entire visual half-field to a dividing line 
which passes through the fixation point, while in other cases a small 
central area of retained vision near the fixation point is left. In double 
hemianopsia two similar sets of cases are likewise observed; both 
halves of the field may be blotted out to the dividing line, causing, of 
course, complete blindness, or a small central area on each side of the 
fixation point may be left, leaving a form of tubal or barrel vision. In 






COETICAL CENTKES 83 

most cases of double hemianopsia one-half of the visual field is ob- 
scured, and later the other. In other words, a lateral homonymous 
hemianopsia becomes, as the result of successive attacks, converted into 
a double hemianopsia, either complete or with a small area of vision 
representing the fovea and its immediate environment. As already 
intimated, the cases of hemianopsia, single or double, with a retained 
area of central vision, are instructive in connection with the question 
of the existence of a separate macular centre in the lower cortical 
visual zone. The cases* can scarcely be explained except on this sup- 
position. 

With regard to double hemianopsia of cortical origin, with which 
I am particularly concerned, cases with lesions strictly limited to the 
cortex can scarcely be found in literature, although in some of the 
few cases recorded the lesions were largely cortical. The case recorded 
by Dejerine and Vialet 13 had several points of particular interest. The 
double hemianopsia, or rather the complete loss of vision, took place at 
once and without any recognizable apoplectiform attack. Peripheral 
and basal disease were eliminated and the diagnosis of cortical blindness 
was made. The patient died after many years and necropsy showed 
bilateral lesions. On the right the lesion involved the cuneus and the 
lingual and fusiform lobules ; in fact, the entire inferior temporo- 
occipital lobe ; on the left the cuneus escaped, but the lingual lobule 
and other portions of the temporo-occipital lobe corresponding to the 
seat of disease on the right were included in the lesion. It is probable 
that the calcarine fissure was implicated. In all cases of double 
hemianopsia in which cortical lesions have been present, these have 
been situated on the mesoventral surface of the occipital lobe, and, as 
far as I know, the calcarine fissure has not escaped in any. The cases 
recorded with necropsy besides that of Dejerine and Vialet, just re- 
ferred to, are those of Schmidt-Rimpler 14 and of Bouveret. 15 

I was consulted with regard to the interestino- case which has been 
recorded by Dunn 16 and which is now part of the literature of the 
subject of double hemianopsia. In this case the patient had loss of 
power of locating or orientating himself. He could not form a con- 
ception of the geography of his house nor of any other place to which 
he had ever been. He could remember a place by its name, but could 
not call it up and relate it with its environment. He retained, how- 

13 Dejerine and Vialet, Centralblatt fur Augenheilkunde, February, 1S94, S. G4. 

14 Schmidt-Rimpler, Archiv fur Ophthalmologie, Xovember, 1SS7. 

15 Bouveret, Revue generale d'Ophtalmologie, Xovember, 1887. 

16 Dunn, University Medical Magazine, Philadelphia. May. 1895. 



84 THE EYE AJSTD NERVOUS SYSTEM 

ever, the ability to recall and to describe individuals. A small central 
area of retained vision remained. Erom a study of his case, Dunn 
suggested the existence in the brain of a geographical centre, where 
are recorded the optical images of locality. It will be noted that in one 
of my schemes of cerebral zones and centres I have placed a centre for 
orientation along the anterior border of the inferior portion of the visual 
field. 

Cases like that of Dunn and a few others which have been reported 
appear to indicate that, in order that loss of orientation shall occur, 
the lesions must be bilateral, that both the right and the left occipital 
or temporo-occipital regions must be the seat of destructive lesions. 
This is not, however, strictly correct. Cases are on record in which a 
destructive lesion of one hemicerebrum caused loss or impairment of 
orientating power. Such a case has been put on record by Peters. 17 
The lesion was right-sided, the patient having left lateral homonymous 
hemianopsia. A lesion situated on either the right or the left side is 
apparently competent to cause impairment or loss of the power of 
orientation, as in another case, that of Groenouw, 18 the hemianopsia 
was for the right fields. It would seem to be much the same with dis- 
order of orientation as with mind-blindness, of which, perhaps, it is 
an expression, — namely, that in the most usual case and most decided 
form it is the result of bilateral lesions, but may occur in special in- 
stances, either transiently or permanently, from a lesion of one hemi- 
sphere. 

Relative cortical hemianopsia is a comparatively rare affection. If 
the theory of Wilbrand were true that light and color are represented 
in superimposed strata, some forms of it would be explicable on the 
view that the most superficial layers of the cortex were destroyed. 
Such destruction might, for instance, cause a hemiachromatopsia, leav- 
ing light perception unaffected, but it is fanciful, if not absurd, to 
reason that any such neatly demarcated destruction of superimposed 
cortical layers takes place as the result of disease, and, so far as I 
know, such a case has never been reported. The manner of blood 
supply to the cortex and subcortex would also make such a lesion un- 
likely. On the theoretical grounds advanced by Wilbrand, that form 
of relative hemianopsia which is known as hemiachromatopsia might 
be explained, but the explanation is not tenable. In the next section 
the probable lesions causing hemiachromatopsia will be considered. 

17 Peters, Archiv fur Augenheilkunde, xxxii. S. 175. 

18 Groenouw, Archiv fur Psychiatrie und Nervenkrankheiten, xxiii. 339. 



CORTICAL CENTRES 85 

Relative hemianopsia sometimes takes the form of a diminution 
of the power of recognizing light, form, and color in the affected field, 
as in a recent case studied by me in which the patient had the general 
symptoms of brain. tumor, such as headache, double optic neuritis, and 
vertigo, with other focal symptoms, besides lateral relative hemianop- 
sia pointing to the left occipital lobe and especially its mesoventral 
surface. In this case the relative hemianopsia eventually became com- 
plete. A relative quadrantic anopsia may be present, and what is still 
more interesting, the case may show in one part of the obscured field 
complete loss of the powers of recognizing light, form, and color, while 
in another the loss may be only for color or form. Of course, if the 
power of recognizing lightness and darkness is lost, color and form 
recognition go with it, but form or color appreciation, or both of these, 
may be abolished and light and dark recognition remain. It may be 
that eventually some clearer insight into the exact position of separate 
centres for light, form, and color will be obtained by a study of cases 
in which there is loss of the three senses in one part of the field, and 
of only one or two in the other. Some curious forms of relative 
achromatopsia will be referred to, in the section on the color sense and 
cortical color centres. 

In the differentiation of cortical anopsias and hemianopsias, as 
well as hemianopsias due to lesions of the optic radiations of Gratiolet, 
the importance of the Wernicke symptom, inaction of the pupils on 
stimulation of the blind half of the retina must be remembered. It 
is only necessary to recall that in cortical, as in optic radiation cases, 
the pupillary response remains for the entire retina, while in cases 
of disease of the optic nerves and tracts and of those portions of the 
basal ganglia which are concerned with vision, the pupillary inaction 
can be demonstrated. Some apparently conflicting cases have been re- 
corded, but these are probably due to imperfect observation. It is 
well known to experienced ophthalmologists and neurologists that it 
is one of the most difficult of undertakings to demonstrate with cer- 
tainty Wernicke's hemianopic pupillary inaction, but nevertheless it 
can be done if sufficient care is taken. In two cases in which Ferrier 
accidentally cut the optic tract in the course of his experiments on 
monkeys, the pupil failed entirely to respond to a pencil of electric 
light carefully focused upon the blind half of the retina, while in a 
number of cases of cerebral extirpation experiments the pupil never 
failed to respond. 

The Wernicke inaction symptom, however, only serves to place the 
lesion in the cortex or the optic radiations. In determining whether 



86 THE EYE AKD KEKVOUS SYSTEM 

the lesion is in the optic radiations or in the calcarine cortex or cuneus, 
the diagnosis must he reached by a study of the associated symptoms 
and of some special features of the hemianopsia. Hemianopsia due to 
lesion of the optic radiations is only in extremely rare cases an isolated 
symptom. Its associated manifestations may be few or comparatively 
many. It may, for instance, be part of a symptom complex which in- 
cludes word-blindness, mind-blindness, and word-deafness, with other 
symptoms so often combined with these forms of sensory aphasia, such 
as alexia, dyslexia, and paraphasia. If the lesion is deeply placed so 
as to implicate the posterior portions of the posterior half of the in- 
ternal capsule, hemiansesthesia may be present, as may also impair- 
ment or loss of the muscular sense and stereognostic perception. When 
hemiplegia or hemiparesis occurs with hemianopsia, which can be re- 
ferred to the optic radiations, the lesion is usually an extensive one, 
although so-called distant symptoms may be exhibited. 

The foregoing are some of the associations by means of which one 
is enabled, with some approach to certainty, to conclude that the lesion 
causing the hemianopsia is subcortical, and probably situated beneath 
and in particular relation with special portions of the lateral aspects 
of the postparietal and temporo-occipital regions. Hemianopsias, cor- 
tical, or largely so, such as were illustrated by the cases of Henschen, 
Holm, Hun, Beevor and Collier, are usually distinguished by the 
absence of such symptoms as hemiansesthesia and the various symp- 
toms classed under the general head of visual and auditory aphasias, 
but if both cortex and subcortex are involved, the associated clinical 
phenomena will depend upon the depth and extent of the lesion. Quad- 
rant anopsia suggests a cortical origin, although not necessarily, as it is 
altogether probable that dorsal, ventral, and medial portions of the 
retinal half -field are represented by separate bundles in the optic radia- 
tions, as they are by subdivision of the cortex in the cuneus and cal- 
carine fissure. 

Dufour 19 has suggested a special means of separating cortical 
hemianopsia from the same symptom dependent upon disease of the 
optic radiations. If the case is purely cortical, he holds that the pa- 
tient will suffer from " vision nulle," that is, from the absence or 
negation of vision. The patient is not conscious that he cannot see 
in the blind field until his attention is called to the fact by perimetric 
or other investigation, or by some accidental circumstance. If the 
hemianopsia is due to lesion of the optic radiations, on the other hand, 

19 Dufour, Revue medicale de la Suisse Romande, August 20, 1889. 



COETIGAL CENTRES 87 

he has a subjective sense of darkness. It is doubtful whether this is a 
reliable test of cortical anopsia and hemianopsia, as a few cases have 
been recorded which seem to be in contradiction to Dufour's views 
with regard to negative vision; in other words, in cases in which the 
necropsy demonstrated the existence of a lesion of the optic radiations, 
Dufour's negative vision was observed. 

Before taking up the subject of cortical color representation and 
chromatic disorders due to disturbance or disease of color centres, I 
shall here make a few suggestions as to terminology which seem to 
me to have some practical value. Hitherto in the discussion of the dis- 
eases of the cortical half-vision centres I have made use of the term 
hemianopsia or anopsia. It might be better, recognizing that the cor- 
tical representation of light, form, and color is separate, to give dis- 
tinct names to the three forms of hemianopsia, — the visual half loss 
for light, for form, and for color. The terms achromatopsia and hemia- 
chromatopsia are well-grounded in medical literature. To express the 
loss of the light sense or the sense of whiteness and blackness, I would 
suggest the word aleucopsia from the Greek, d, priv. ; Xsux6$ y white, 
and hemialeucopsia for the loss of this sense in the visual half fields ; 
for the loss of the sense of form the term amorphopsia, from the Greek 
d } priv.; ^opvrj , form, and for this loss in the corresponding half 
fields, hemiamorphopsia. These terms may be found convenient in 
description. It is true that when the loss of the light sense is complete 
that of form and that of color necessarily go with it, but as was seen 
when discussing relative hemianopsias, we may have incomplete forms 
of hemialeucopsia. 

Hemialeucopsia has received the consideration required in this 
-chapter in the discussion of hemianopsia in its cerebral and especially 
its cortical relations, and the achromatopsias will be presently con- 
sidered. A few words might be said rather in the way of suggestion 
than of detailed consideration about the study of amorphopsia, espe- 
cially with reference to its bearings upon questions of cerebral localiza- 
tion. Usually the methods of examining the fields for form are of the 
simplest character, although sufficient for the ordinary purposes of the 
ophthalmologist. The reader unacquainted with these methods is re- 
ferred elsewhere and can find them in the works on ophthalmology. 
An adaptation of the eye to different degrees of illumination is re- 
quired for a better study of these fields, and particular methods of get- 
ting the best results have been described by some writers. 

I have already expressed the view that the only primitive cortical 
centre of vision is that for light or for the recognition of whiteness 



88 THE EYE AND NERVOUS SYSTEM 

and blackness, and that color recognition develops ventrally in the 
expansion of the cerebral surface, the recognition of form probably 
developing dorsally. In the diagrams, Figs. 5 and 6, the most ele- 
mentary centres for form are represented as probably situated along 
the edge of the hemisphere, both on its mesal and lateral aspect; in 
other words, in the most dorsal part of the cuneus and in the first 
occipital convolution. Presumably elementary forms are represented 
cortically in closest relation with the primitive centre for light, the 
representations for more and more complex forms receding with cor- 
tical development from the primitive area of representation. 

Little is known about form fields through studies of their extent by 
means of objects of different shape and character, and it is possible that 
something of value with regard to cortical localization might be learned 
by perimetric observations of the fields for form as determined with 
articles of particular shape or design, instead of confining these in- 
vestigations to the use of a limited number of geometric figures or to 
letters in one or two languages. These studies might prove of par- 
ticular value in cases of partial mind-blindness or of word- or letter- 
blindness with large retention of vision for other purposes. 

CEREBRAL CHROMATIC PHENOMENA AND THE COR- 
TICAL CENTRES FOR COLOR RECOGNITION. 

Color-blindness, or achromatopsia, is the inability to recognize col- 
ors. It may be total, although this form of achromatopsia is rare, and 
even when it is present the individual may distinguish as differences 
in lightness or darkness what others recognize as differences in color. 
Most frequently the color-blindness is partial, and this partial color- 
blindness may be of several forms. Hemiachromatopsia is the loss 
of the ability to recognize colors in one half-field. Central achroma- 
topsia is an affection in which the half visual fields retain the power 
of color recognition, while this is lost in the central or macular field. 
Dyschromatopsia is difficulty in distinguishing colors, and parachro- 
matopsia is false or incorrect perception of colors. Partial color- 
blindness may be regarded from another point of view, as to the color 
or colors which are lost or disturbed ; this is illustrated by such affec- 
tions as red-blindness, green-blindness, violet-blindness, or red-green 
blindness. Color amnesia, or color aphasia, sometimes incorrectly 
spoken of as amnesic color-blindness, is an affection in which the 
patient, while able to recognize colors by the different tests, is unable 
to name the colors recognized. 

For the sake of a clear understanding of cerebral color affections, 



COKTICAL CENTRES 89 

it may be worth while to call attention here to a few well-known facts 
regarding chromatic phenomena. The sensations of color result from 
dissolving white light into its spectral colors. The so-called primary 
colors of the spectrum, as green, red, yellow, and blue, each includes 
somewhat differing sensations of color. These correspond roughly to 
what are known as shades of color. The power of recognizing these 
differences varies greatly in different individuals, and even in the same 
individual at different stages in the course of his education or train- 
ing. The mixing of certain colors together and the mixing of certain 
colors with white light produces new sensations of color. A word or 
two might be said about a few of the terms frequently used in the 
discussion of color }menomena. Hue depends on the wave lengths 
of the rays; the intensity on the amount of light falling on a body 
in the given time, and the saturation on the amount of white light fall- 
ing on the area in the same time. Mixing of the colors of the spectrum 
produces white light, or the same can result from the mixture of com- 
plementary colors, although in this case the light will differ in intensity 
from the white light which is the effect of mixing all the colors of the 
spectrum. 

By hue we mean color in the ordinary sense in which the word is 
used ; it is hue .which gives red, green, blue, etc. The hue, however, 
is not always simple and therefore cannot always be defined by making 
it synonymous with wave lengths. It may be the result of an admix- 
ture with various spectral colors, or of white with some colors, and then 
it can only be expressed by averaging or approximating the wave 
lengths of the spectral colors. Sometimes the three constants of color 
are defined as hue, purity or tint and brightness. Purity or tint does 
not depend upon the wave length, but upon the amount of white light 
that enters into the colors ; the less there is of white light the purer 
is the color. 

Brightness depends upon the energy of the ethereal movement and 
the sensitiveness of the retina for the particular color. Explained in 
another way, the brightness is in inverse proportion to the amount of 
blackness mixed with the color. In the present connection we are 
only concerned with these general phenomena of color in so far as 
they have some bearing upon the question of cerebral activity with 
regard to color sensations. Hue, intensity, tint, and brightness as sub- 
jective phenomena all have their cerebral correlatives, but the only 
constant material substratum is for hue as expressed in fundamental 
colors. 

It has been discovered that in the retina exists (me and perhaps 



90 THE EYE AND NERVOUS SYSTEM 

several photo-chemical color substances. The best known of these is 
the visual purple. The existence of this substance has been demon- 
strated in various ways, as by removing the eyes of a frog after having 
kept the animal some time in the dark, when the retina is found to be 
of a red or purplish color, which soon fades, leaving it an opaque white. 
Investigation has shown that this visual purple is confined to the rods 
and even to their outer limbs. It is absent from the fovea centralis. 
It is not present in the eyes of some animals, as, for instance, in the 
eyes of snakes, whose retinae are composed almost entirely of cones. 
It is believed, but not fully demonstrated, as in the case of visual pur- 
ple, that in the retina are present visual substances for each of the 
fundamental colors. 

As is well known, views differ as to which of the colors of the 
spectrum are fundamental. Helmholtz believed that they were three 
in number, — namely, red, green, and violet; while, according to 
Donders, red, yellow, green, and blue were primary or simple colors. 
Hering regarded as distinct the recognition of white, black, red, yellow, 
green, and blue. Other views as to the fundamental colors have been 
held, but these are usually represented as three or four in number. 
The hypothesis of three fundamental colors was first put forward by 
Young. Foster and many physiologists accept the trichromatic view; 
all agree that while there may be more, it is necessary to recognize at 
least three primary colors. Each of these probably has its own visual 
substance, so that in the retina, besides the visual purple, there is 
probably a visual red, visual green, visual blue, and possibly a visual 
yellow substance. * 

The theory is that the decomposition of each visual substance is 
accompanied by molecular vibrations which are taken up by the retinal 
structures and transmitted through the various layers of the retina 
to the optic nerve, and thence by the optic tract, basal centres and 
optic radiations to the visual cortex. The colors are sifted at the 
periphery of the visual apparatus and each sifted color has its place 
of representation in the cerebral cortex. 

Young held that the three primary color sensations were repre- 
sented by three distinct nerve arrangements in the retina and visual 
apparatus. Whatever view is taken as to the number and character 
of the fundamental colors, I believe with Young and Helmholtz that in 
the peripheral end organs are separate structures for their sifting. It is 
with colors as it is with other forms of sensation. All cutaneous sensa- 
tions, as, for instance, those for touch, pain, and temperature, undergo 
a receiving and sifting or differentiating process at the body's pe- 



CORTICAL CENTRES 91 

riphery. These sifted sensations are carried to the dorsal ganglia, to 
the spinal cord, to the oblongata, the basal ganglia, and the brain cor- 
tex by separate tracts, and it is believed that they have separate centres 
of representation in the cortex. The phenomena of dissociated cu- 
taneous sensations are best explained on this supposition, and color sen- 
sations are dependent upon analogous structures and processes. 

Colors are recognized by means of a specially developed apparatus, 
which, beginning with certain photo-chemical substances in the retina, 
ends with cell groups located in a part of the cerebral cortex set apart 
for color recognition. In the retina are differentiated structures for 
the sifting of different colors, and midway between these and the ulti- 
mate cortical centres for colors is a basal relaying station, the pregenic- 
ulum, in which colors receive their first central differentiation. Trans- 
mitting structures for color sensations, probably also differentiated, are 
contained in the optic tract and the optic radiations. It is through the 
workings of this barely sketched complex mechanism that colors are 
appreciated as separate sensations. The first sifting takes place in the 
retina, the last in the cortex, and blendings as well as siftings occur 
in both. These blendings give the different hues and shades of color, 
those in the cortex being interpreted through cellular associations. 

Before taking up the consideration of cortical disease causing color 
impairment or loss, it will be useful to glance at the various focal affec- 
tions of the entire visuo-chromatic apparatus capable of producing these 
affections. Beginning at the extreme periphery, in the first place, one 
or more of the photo-chemical substances in the retina may be congeni- 
tally absent or deficient, or these may be injured or destroyed. Our 
knowledge is, however, quite limited as to achromatopsias, which have 
their cause in abnormalities of the color substances; in the second 
place, disease involving the peripheral nerve apparatus, which takes 
part in the early stages of color sifting, that is, of the rods and cones 
and of the ganglia-cells or other cells of the retina, may cause color- 
blindness or some form of disturbance of color perception. This has 
been observed, for instance, in some of the forms of retino-chorioiditis 
involving either the macula or the retinal periphery. 

Color sensation may be interfered with or lost by any form of 
disease or disturbance which impairs or destroys the conducting fibres 
concerned with the transmission of those retinal stimuli which give 
rise in consciousness to. ideas of color. The optic nerve and tract arc 
subject to such affections as retrobulbar neuritis in toxic amblyopias, 
and neuritic atrophy in tabes dorsalis. They and the optic radiations 
may be the seat of embolic or thrombotic lesions causing softening, or 



92 THE EYE AKD NERVOUS SYSTEM 

somewhere along these transmitting paths may be located hemorrhages, 
abscesses or tumors. In so far as such lesions interfere with the fibres 
or fibrils, whose business it is to transmit vibrations concerned with 
color perception, impairment or loss of the power of recognizing colors 
will result. Gross or microscopic disease of the pregeniculum, the great 
relaying station in the visual pathway, will give various affections of 
the color sense, according to its nature, exact position and extent. 

In the occipital cortex are situated separate centres for the recogni- 
tion of the fundamental colors. Authorities equally high differ with 
regard to this subject: some, like ITolden 20 in a recent paper, arguing 
that it is not necessary to predicate the existence of cortical color cen- 
tres in order to explain the normal chromatic phenomena or the dis- 
turbances in color recognition which result from disease or injury of 
the brain; others, like Landolt, Verrey, 21 and Vialet, with whom I 
agree, believing that distinct centres for luminosity, form, and color 
are differentiated in contiguous regions of the cerebral surface. Pro- 
visionally I would place these centres of chromatic representation ven- 
trally to the primitive centres for light, either in the lingual lobule if 
the representation of light, that is, of whiteness and blackness, reaches 
only to the calcarine fissure, or in the fusiform lobule (fourth temporo- 
occipital convolution) if the primary centre includes, as some sup- 
pose, the lingual lobule (fifth temporo-occipital convolution). 

It would, of course, be absurd to contend that the almost innumera- 
ble shades and tones of color have separate representation in cerebral 
cortical cell groups. The representation as indicated is probably only 
for the fundamental colors. What is known as to complementary colors 
and as to the results of the admixture of different colors in the produc- 
tion of white or gray, and also as to the blending of white or black with 
the various fundamental colors, suggests the probable explanation of 
the cerebral processes related to the recognition of the numerous shades 
and tints of color. Both in the eye and in the brain fine color dis- 
crimination is brought about through processes of blending or associa- 
tion ; at the periphery through photo-chemical blendings and end-organ 
selection, in the cortical centres through neuronal and neuro-fibrillary 
associations. 

Wilbrand's 22 hypothesis regarding the centres for color and for 
light and darkness, already alluded to, is well known and of much 






20 Hoklen, Archives of Ophthalmology, vol. xxiv. p. 447. 

21 Verrey, Arch. d'Ophthal., par. v. 8, July and August, 1888. 

22 Wilbrand, Ophthalmiatrische Beitrage zur Diagnostik der Gehirnkrankheiten, 
1883. 



COKTICAL CENTRES 93 

interest and needs fuller consideration here. He would have us be- 
lieve that the cell groups representing color and light are placed in 
layers or strata, one above the other, in the cerebral cortex of the 
calcarine fissure. Stratification localization has been favored by not a 
few neurophysiologists. The re}3resentation of the tactile sense, the 
muscular sense, and of movements have, for instance, been regarded as 
in superimposed layers in the recognized motor cortex. The methods 
of the evolution of cerebral structure are opposed to views of this kind. 
The cells and cell groups which minister to special functions and to 
association are arranged with considerable uniformity in a similar 
way in layers and strata in all parts of the brain. As function is 
added to function and as function is modified in the course of phylo- 
genetic and ontogenetic evolution, the unvarying tendency in anatom- 
ical development is towards surface expansion. To accommodate 
evolving function new regions of the cerebral cortex are added. In 
this way primary, secondary, and tertiary fissures and gyres have de- 
veloped. The organ of vision, peripheral and cerebral, at first only 
capable of appreciating light or lightness and darkness, as evolution 
advances, acquires the power of recognizing color and form. 

According to Wilbrand, the light-dark or white-black centres are 
located in the deepest layers of the primary visual cortex near the 
centrum ovale, while the cells representing the different colors are 
arranged in superimposed strata nearer the cortical surface. In order 
that sensations of color shall be realized, it is necessary, according to 
him, that sensations of lightness and darkness shall be first appreciated, 
and for this reason these perceptive centres are most deeply placed in 
the cortical cinerea. A visual sensation of light having occurred, this 
is transferred to superimposed layers. Wilbrand applies Hering's 
valence theory as to the manner in which atoms combine, to explain 
what occurs in the process of the recognition of colors. The percep- 
tion of color is in accordance with the valence relations which the 
colors bear to lightness and darkness. Red, green, blue, yellow, each 
of the fundamental colors, has in it its own Color valence and besides 
a certain valence of white light, and in accordance with the destruction 
or disturbance of this valence will be the degree of impairment of 
color vision. The perceptive centres for light and colors in the cor- 
tex have a power of valence differentiation which corresponds with 
that possessed by the photo-chemical substances and peripheral nerve 
structures at the other extremity of the visuo-chromatic cerebral 
apparatus. 

I shall next take up the question of the position of the centres of 



94 THE EYE AND NEKVOUS SYSTEM 

color representation from the standpoint of clinicopathological evidence. 
The cases put on record are chiefly illustrations of loss of color recog- 
nition in the half -fields; in other words, of hemiachromatopsia. Cen- 
tral or paracentral achromatic scotomata are sometimes observed, and 
doubtless with closer observation we shall in the future be able to 
determine central as well as peripheral fields of color recognition with 
separate cortical centres for both. It is altogether probable also that 
for color as for light there are lower and higher macular centres, but 
we are not yet in position to demonstrate this fact. 

The clinicopathological evidence as to the position of the centres 
of color recognition in the cortex is meagre and of an unsatisfactory 
character. It favors, however, the convolutions situated ventrad of the 
calcarine fissure as its probable seat. In a case reported by Henschen 2S 
the lingual and fusiform lobules were involved in the destructive lesion, 
although, unfortunately for the purposes of argument regarding the 
color centres, other portions were also implicated. 

Two of the most interesting cases of achromatic defect with lesions 
of the brain demonstrated by necropsy are those of Brill and of Verrey. 
As the case of Brill, 24 although reported twenty-two years since, has 
received little or no attention from writers on the subject, I shall refer 
to it in some detail. 

The patient, a man sixty-three years old, in October, 1881, had an 
apoplectic attack resulting in right hemiparesis, right hemiansesthesia, 
incontinence of urine, and increased right knee jerk. After several 
weeks these symptoms were changed and supplemented ; the anaesthesia 
of the thigh became hyperesthesia, tremor of the paralyzed side de- 
veloped, and the patient suffered from headache, restlessness and in- 
somnia, with spells of tinnitus and dazing and also dulness of mind. 
His sight became dull and hazy. Four months after the attack- the 
patient was examined by Dr. Brill and Dr. E. C. Spitzka in consulta- 
tion. He failed to recognize green ; he was doubtful as to whether vio- 
let was greenish or bluish ; green, he said, was red or rather pinkish ; 
he could always recognize red and yellow. The color loss involved both 
eyes. Gross objective vision was retained except a subjective blurring; 
the fields of vision were not limited. The patient was illiterate, so that 
his condition as to letter-blindness and word-blindness could not be 



- 3 Henschen, Klin. u. Anat. Beitr. z. Pathol, des Gehirns, Upsala, 1890, 1903. 

** Brill, Amer. Jour, of Neurology and Psychiatry, vol. i, N. Y., 1882. Previous 
to the report of the necropsy, Brill had reported this case clinically in the Chicago 
Med. Review, April, 1882. 



CORTICAL CENTRES 



95 



determined. He had worked on the railroad for thirty years before 
his illness, and was able to recognize all colors. 

He died about six and a half months after admission to the hos- 
pital. At the necropsy a softened area was found at the apex of the 
left cuneus and the adjoining upper part of the lingual convolution. 
Brill exactly describes the area of softening as follows : " The focus 
was triangular, the apex being directed forward and the short side 
posteriorly. It extended to within a centimetre and a half of the apex 
of the occipital lobe, and involved half the altitude of the cuneus in 
the middle of the latter and almost the entire apex of this lobe. The 
gyrus lingualis was involved for about one-quarter of its width on the 
surface, and evenly from the posterior almost to the anterior extent 
of the lesion described (Fig. 14). The calcarine fissure could not be 




Fig. 14. — Mesal face of the occipital lobe, showing- the superficial position and extent of an area 
of softening causing achromatopsia ; the calcarine fissure is indicated where the white interspaces are 
shown ; the lesion involved the inferior portion of the cuneus and a bordering zone of the lingual 
lobule. (Brill.) 

identified excepting its most anterior extremity, and even at this 
spot both borders were softened. At its lowest border the softening 
reached almost to the posterior cornu." Brill cites chiefly from Ex- 
ner's 25 work on cerebral localization a number of cases of impairment 
or loss of color recognition from cerebral disease. 

It is unfortunate that Brill in the report of this most interesting 
case does not clearly indicate whether the color-blindness in both eyes 
was hemiachromatopsia or an absence of the power of recognizing 
colors without reference particularly to the half-fields. Nevertheless the 
case is of value in connection with the necropsy, which clearly iiuli- 



25 Exner, Untersuchungen fiber die Localization der Functionen in der Grosshirn- 
rinde des Menschen, Wien, 1881. 



96 THE EYE AND NERVOUS SYSTEM 

cates that cerebral achromatopsia in this, as in the other cases referred 
to, was associated with disease of the region ventrad to the calcarine 
fissure, although it also included this fissure and a part of the cuneus. 

Verrey reported, in 1888, the most interesting case of hemiachro- 
matopsia with necropsy, showing the lesion to which the loss of the 
chromatic sense was due, which has yet been put on record. The 
patient was a woman sixty years old, whose symptoms were difficulty 
in reading, with good general visual acuity, slight concentric contrac- 
tion of the field, complete absolute right hemiachromatopsia, and a 
sensible diminution of visual acuity for the right half of the visual 
field. The patient had paroxysmal headache and attacks of vertigo 
with vomiting. She gradually developed fatigue in reading, with letter 
dancing and dyslexia. Examination showed that with her central field 
she could read letters and words written in different colors. Twenty 
days before her death the patient's symptoms were much the same as 
above summarized, with the exception of a further enfeeblement of 
luminous perception. Eight days before her death she fell, and at 
this time developed a complete left hemiplegia, entire cutaneous irre- 
sponse, violent vomiting and clonic and tonic convulsions of the face 
and right side. The necropsy showed recent large hemorrhage in the 
right hemisphere destroying the centrum ovale and the basal ganglia, 
and filling the right lateral ventricle. A fresh hemorrhage the size 
of a bean was situated at about the junction of the anterior and middle 
third of the left callosum. A second lesion was found between the 
floor of the posterior horn of the left lateral ventricle and the basal 
surface of the occipital lobe. Close examination showed a large throm- 
bosed vessel, and that the parts destroyed were the white substance of 
the third occipital convolution, more or less completely the white sub- 
stance of the occipital extremities of the lingual and fusiform convolu- 
tions, and the posterior inferior point of the cuneus. The superficial 
involvement of the lingual and fusiform convolutions is shown in 
Eig. 15. 

The following case of double hemiachromatopsia, recorded by Mac- 
kay and Dunlop, 26 also throws some light on the probable position of 
the cortical color centres. The lesions present were in the temporo- 
occipital lobe almost entirely ventrad of the calcarine fissure. The 
patient, a man sixty-two years old, had a sarcoma of the stomach. He 
had an attack of general disturbance of vision, and examination on the 
same day showed that he was completely color-blind. Examination 

28 Mackay and Dunlop, Scottish Med. and Surgical Journ., December, 1899. 



CORTICAL CENTRES 



97 



showed a double homonymous hemianopsia with greater involvement 
of the left side. Later the power of vision for light and form improved, 
while that for color remained permanently lost. Eive months after the 
first attack causing disturbance of vision and color-blindness he had an 
attack of right hemiplegia from which he died. At the necropsy the 
optic nerves were found to be intact. Both occipital lobes showed 
atrophic areas. The atrophy included the posterior parts of the tem- 
poro-occipital convolutions on both sides with an extensive destruction 
of the tissues on the right side. On the left side the atrophy extended 
deeply and involved a small area of the gray substance of the cal- 
carine fissure. On both sides the lower borders of the optic radiations 




Fig. 15.— Mesal aspect of the left hemicerebrum, showing the cortical extent of a lesion causing 
liemiachromatopsia ; the subcortical radiations of the lesion are described in the text. A small lesion 
of the callosum is also shown. (Verrey.) 

were affected. The process was supposed to be due to hyaloid de- 
generation of the vessels. The case was not a pure one of liemiachro- 
matopsia ; the visual fields for light and form were to some extent 
affected. 

In this case the chief destruction causing the hemiachromatopsia 
was in the lingual and fusiform lobules (fourth and fifth temporo- 
occipital convolutions) and the posterior inferior portion of the cuneus 
and calcarine fissure were implicated. This case again points to the 
convolutions situated ventrally to the calcarine fissure as the part of 
the temporo-occipital region most probably concerned with color rec- 
ognition. 

A case recorded by Swanzey 27 carries with it some suggestion as 
to the site of the centres for color recognition. A man seventy-seven 

27 Swanzey, Trans. Oph. Soc, vol. iii. p. 185. 

7 



98 THE EYE AND XERVOUS SYSTEM 

years old suddenly became affected with a slight homonymous defect 
for light in the left upper quadrant of the field of vision, while over 
the entire left field color sensation was absent. This case, as pointed 
out by Gowers, suggests that the centre for colors is adjacent to that 
part of the half vision centre in which the upper quadrant is repre- 
sented. It will be remembered that the case of quadrant anopsia of 
Beevor and Collier seems to sIioav that the upper quadrant of each 
visual field is represented in the lower part of the cuneus and the 
calcarine fissure and perhaps in the lingual lobule. Tf this be true 
the case of Swanzey would point to the parts adjoining this quadrantic 
centre, most probably to the lingual or fusiform lobule as the seat of 
color representation. 

A few other cases of achromatopsia probably due to cortical, or at 
least to cerebral, disease are worthy of citation, although little is 
taught by them with regard to the position of the color centres. In a 
case reported by Eperon, 28 a man seventy years old had suffered with 
hemiparesis, which almost entirely disappeared except a sensation of 
numbness in the right arm; suddenly he developed word-blindness. 
On examination it was found that in the entire right half of the field 
the sensation for color was missing. Perception of light and acuteness 
of vision, although present, were considerably reduced. A man fifty- 
four years old had diminution of vision with glimmerings or flickerings 
before the eyes for ten or twelve days. He had complete left hemian- 
opsia for all colored objects ; he also had hemianopsia for small white 
objects, but not for those of larger size. His vision was five-sixths of 
the normal when black characters were on a white ground, but when 
the characters were pale the vision was reduced to six and a half. Im- 
provement took place, but symmetrical scotomata remained on each 
side, about fifteen millimetres from the point of fixation. Sensation for 
colors returned, but not as distinctly as before the attack. After this 
decided improvement a sudden right-sided hemiansesthesia appeared 
and vision became worse again. A small defect for white was present 
in the whole of the upper right quadrant. This case is recorded by 
Meisling. 29 In another case, which we owe to Ziehl, 30 a man on awaken- 
ing in the morning found that he was unable to read. Hemianopsia 
was absent, but right homonymous hemiachromatopsia was present, 
the dividing line passing vertically through the fixation point. In the 

^Eperon, Archives d'Ophthalmologie, iv. 1884, Juillet-Aout. 
* 9 Meisling, Ref. Jahresb. f. Ophth., 1897, p. 314. 

80 Ziehl, Verhandl. d. Gesellsch. Deutscher Naturf. u. Aerzte. 67. Vers, zu Liibeck. 
ii. Teil.; ii. Halfte, p. 184, 1805. 



CORTICAL CEXTRES 09 

affected fields all colors appeared as gray. In a case recorded by 
Gaudenzi, 31 relative hemialeucopsia and hemiachromatopsia were 
present. 

In a case of hemiachromatopsia, reported by Samelsohn, 32 the 
autopsy showed a gliosarcoma of the optic tract with some invasion of 
the thalamus and geniculate body, the optic radiations and cortex being 
free from disease. In a case of IThthoff, 33 with normal sharpness of 
vision, unilateral hemianopsia for color and normal limits for white 
were present, the autopsy revealing a sarcoma of the pons which com- 
pressed downwards the cerebral peduncles and reached into the third 
ventricle. On the left the tumor had grown into the anterior portion 
of the median fossa causing protrusion of the dura. The chiasm was 
much attenuated. The right tract was somewhat contracted in the 
position of the chiasm. In another case put on record by Samelsohn 34r 
the patient had some difficulties of vision following an apoplectiform 
seizure. At this time the entire right side was paralyzed, but when 
examined by Samelsohn only a slight hemiparesis of the right arm and 
thigh without any disturbance of sensation could be found. The pa- 
tient had a typical left-sided hemianopsia for colors. From the vertical 
dividing line all colors were seen on the right, but none on the left. 
Bjerrum 35 has reported the case of a man thirty-nine years old, who, 
after headache lasting fourteen days, developed indistinct vision. The 
outer limits of the light field were normal, as was also the field for 
form. Upon examining him with colored objects it was found that he 
was suffering with complete color-blindness in the left half of the visual 
fields for both eyes, the dividing line being vertical to the fixation 
point. All colors in the achromatopsic fields were recognized as gray. 
The day after he was examined intense headache and inflammatory 
swelling in the left temporal region were present. An abscess formed; 
an incision was made, and the patient died, but necropsy was refused. 

Besides the case of Mackay and Dunlop, already referred to, a few 
other cases of double hemiachromatopsia have been recorded. Steffan 36 
recites the case of a man sixty-two years old, a printer of colors, who, 
before the attack, having had a perfect sense for colors, after an 
apoplectiform attack with dizziness and darkness before the eyes, bur 

31 Gaudenzi, Ref. Jahresb. f. Ophthalm., 1899, 476. 

3 - Samelsohn, Ein Fall von Hemiachromatopie. Berliner klin. Wochenschr., 
1890, p. 331; Niederrhein. Gesellsch. zu Bonn. 

33 Uhthoff, Berliner klin. Wochenschr., Nr. 12, 1897. 

34 Samelsohn, Centralbl. f. Wissensch., 1881, Nr. 47 u. 50. 

35 Bjerrum, Ref. Centralbl. f. prakt. Augenheilk., 1881. 471. 

36 Steffan, Arch. f. Ophth., xxvii. 1881. 

Lor a 



100 THE EYE AND NERVOUS SYSTEM 

without paresis or loss of consciousness, had total color-blindness in 
both eyes without affecting the sharpness of vision or the visual fields. 
Examination of the eyes showed nothing abnormal. Examinations 
were made shortly after the attack and also four years later. At this 
time he was not totally color-blind, but tests showed pronounced blind- 
ness for red and green and diminution for other colors. The central 
sharpness of sight and the visual fields were still normal. No dis- 
turbance was present of the sense of white or black. Later on the sen- 
sation for red, yellow, and blue was present, but only when the colors 
were extremely intense. Alexander 37 reports the case of a man who 
had, a short time before coming under observation, lost the ability to 
recognize colors. On examination with samples of colored paper, light 
green appeared to be yellow, dark gray violet, red, and scarlet and 
brown, green. The acuity of vision was normal. The eyegrounds were 
normal, with the exception that they were considerably pigmented. 
Two years later the condition had changed but little. The color-blind- 
ness was extended over almost the entire visual fields. No scotomata 
were present. A man fifty-four years old, whose history is given by 
Quaglino, 38 after an apoplectiform attack was found to be completely 
amaurotic and paralyzed on the left side. The hemiplegia disappeared 
slowly and the visual power gradually improved. In a year the hemi- 
plegia had disappeared entirely. The visual acuity was now excellent, 
he being able to read the smallest type and see the small birds at the 
top of the trees. He had, however, incomplete left lateral homonymous 
hemianopsia. After the attack he could not recognize any colors but 
black and white; all faces appeared to him pale and colorless. The 
color-blindness was not confined to the hemianopsic field, but 
extended over the fields of both eyes. This patient lost the ability to 
recall the form and configuration of objects, although he knew what 
they were when they were first seen by him. In a case of Schoeler and 
Uhthoff, 39 a man sixty-two years old, in full possession of the color 
sense, developed a right-sided hemiachromatopsia. He also had upper 
quadrantic achromatopsia in the opposite fields. It will be seen that 
this was not a complete double hemiachromatopsia. 

In some cases in which the color sense is affected a person can dif- 
ferentiate between different colors, and yet not have the power to pick 
out particular colors. He may recognize the fact that red differs from 

37 Alexander, Arch. f. Ophth., xv. iii., 102. 
as Quaglino, Giornale d'oftalmologia, 1867. 

39 Schoeler and Uhthoff, Beitrage zur Pathologie der Sehnerven und der Netz- 
haut., Berlin, 1884; H. Peters, p. 88. 






COKTICAL CENTKES 101 

green and green from blue or yellow, or that any one color from one 
or all other colors, and yet he may not be able to say which is this or 
that color. In a case of Lissauer 40 the patient could differentiate be- 
tween colors in wools, but when asked to pick out special colors he made 
the grossest errors. With his color-blindness a diminution in visual 
acuity was associated. 

Under such names as color amnesia and amnesic color-blindness is 
described a symptom which is exhibited by the inability of the person 
to name a color which he evidently recognizes. It is a variety of the 
visual anomia (word-dumbness, optic aphasia) hereafter described, see 
page 148. The patient may be unable to name any color, or his word- 
dumbness may be for only one or two colors. In a case recently stud- 
ied by me, for instance, when a green testing object was presented to 
the patient, he could not name the color, although indicating that he 
knew it. Finally he said, "It is the Irishman," showing in this way 
his appreciation of the fact that it was green. He instantly recog- 
nized blue and red ; yellow he called a " dirty white." The same 
answers were given for either the right or the left eye and for both 
eyes together. When he was given a number of objects of different 
colors and asked to pick out particular colors, he always did so cor- 
rectly, thus differing from the patient of Lissauer, above referred to, 
who could not do this, although he knew that the colors were different. 
This symptom should, I believe, be termed color aphasia or color 
anomia rather than color amnesia or amnesic color-blindness. It is 
probably dependent upon some disturbances of the tracts associating 
the cortical centres for color with the speech centres, sensory or motor. 

Some of the hysterical disorders of the color sense are of particular 
interest in connection with the discussion of the question of cortical 
centres for color, both in the lower and in the higher visual fields. The 
mere fact that these disorders are nearly always, if not always, uni- 
lateral, points to unilateral cerebral centres of representation. It 
is not within the scope of this article to direct attention in detail 
to this subject, which is considered in chapter xvi. I would like, 
however, to point out in this connection that the most probable ex- 
planation of the phenomena is similar to that which is given for 
unilateral amblyopia when discussing the crossed amblyopias. The 
visuo-sensory centres for color are functioning normally, or in condi- 
tion to so function. The visuo-psychic centres for color are in abey- 
ance or are temporarily functionally disjointed from the primary cen- 

40 Lissauer, Archiv fur Psychiatric und Ncrvcnhcilkurulc. vol. xxi, 1SS0-1S90. 



102 THE EYE AND NERVOUS SYSTEM 

tres. The affection is psychic, as Bernheim 41 believes. The same ex- 
planation holds as would be given for various phenomena of hypnosis. 
A few remarkable cases of total uniocular color-blindness have been 
put on record, the acuity of vision and the fields for light and form 
being normal. The difficulty of explaining such cases on the doctrine 
of cortical arrest or destruction is evident. The centres for color, like 
those for light, are not for each eye, but for the homonymous fields of 
both eyes; and it is therefore necessary to believe that the centres 
related to the direct fibres of the optic path on one side and those 
relating to the crossed fibres on the other side are arrested in develop- 
ment. This, although improbable, is by no means impossible. Con- 
genital arrest may depend upon bilateral defect of centres which work 
together in function. Uniocular color-blindness is better explained on 
the theory of deficiency or disease in front of the chiasm, as, for in- 
stance, absence of the color substance in one eye, or unilateral defect 
of the peripheral nerve organs concerned with color selection. In some 
cases this unilateral color-blindness is partial ; it may, for instance, 
be for red-green or blue-yellow. In bilateral cases of congenital color- 
blindness, which is the most common form, the powers of visual recogni- 
tion for light and form are retained, but all colors fail to be recognized. 
Such a total congenital color-blindness may be due to the arrested de- 
velopment of the entire apparatus concerned with color sensation; or 
of some one portion of the apparatus, as of its periphery, of its basal 
centres, or of its cortical centres in their entirety. 

AMBLYOPIAS DUE TO DISEASE OF THE VISUAL CORTEX OR 
OF ITS ENTERING AND CONNECTING TRACTS. 

Besides the anopsias, hemianopsias and achromatopsias described 
as the results of lesions located in certain portions of the areas for 
vision of the cerebral surface, other forms of impairment of sight, due 
to disease of the visual cortex, or of its entering or connecting tracts, 
are sometimes met with and are usually included under the general 
head of amblyopias. Crossed amblyopia is an affection of sight due to 
cerebral disease, in which the sole or chief impairment is on the side 
opposite to the lesion. In the few organic cases of this disease which 
have been recorded, moderate or slight dimness of vision on the same 
side as the lesion has been present in connection with the marked con- 
tralateral impairment or loss. 

41 Bernheim, Brain, vol. 16, 1893. 



CORTICAL CENTRES 103 

The discussion of the subject of crossed amblyopia is intimately 
related to that of cortical macular representation. I have already indi- 
cated my belief in the existence of an area of macular representation 
in the primary or lower visual centres, and I have subscribed for the 
present to the view of Henschen that this macular centre is probably 
situated in and around the anterior extremity of the calcarine fissure. 
In the higher visual zone, in the angular or angulo-occipital region, is 
also situated a cortical area for the macula. The lower macular area 
subserves clear vision in what might be termed its most primitive form. 
Luminosity, or, rather, whiteness and blackness, are here perceived ; ob- 
jects here first receive their clearest recognition. The centre, however, 
is purely perceptive ; it is simply a part of the visuo-sensory area of 
Campbell and Mott. Natural objects, faces, persons, designs, words, 
letters or numbers are perceived through the functioning of this lower 
macular cortical area, but without a recognition of their significance; 
they are perceived, but they are not apperceived. Perception by the 
lower macular centre does not evoke concrete ideas the result of visual 
memories. Such visual ideas are the outcome of the activities of the 
higher visual field, including its macular subdivision. 

It may be well to direct attention here to the different views which 
have been held with regard to macular representation. While Hen- 
schen, whom the writer had chosen to follow, would limit it to the 
anterior and ventral portion of the cuneo-calcarine visual centre, others 
have suggested as its location the posterior extremity of the calcarine 
fissure and its bordering cortex. Von Monakow holds that the macula 
has a variable representation spread out over both the lateral and mesal 
aspect of the occipital lobe ; in other words, over both the lower and 
higher visual fields. The facts at our command do not seem to me 
to support this view. The physiology of sight and clinical experience 
point to a more or less concentrated cortical representation of the 
macula. 

From his own experiments and those of Schafer and Munk, Ferrier 
argues that the angular gyres are more particularly related to the areas 
of distinct vision, — that is, that they represent the macula. He also 
holds that the macula is represented in both hemispheres, but most in 
the angular convolution of the opposite side. Llis views are indicated 
schematically in Fig. 16. 

Lannegrace 42 and Ferrier and Yeo, who have performed numerous 
extirpation experiments on the occipital lobe and the angular gyre of 

42 Lannegrace, Influence des Lesions Corticales sur la Vue, Archives de Medecine 
Experimental, 1889. 



104 



THE EYE AND NERVOUS SYSTEM 



the brains of monkeys, hold that destruction of the occipital lobe causes 
no appreciable impairment of vision, while destruction of the angular 
convolution causes crossed amblyopia of a temporary character. In 
one of Ferrier's most striking experiments he removed one of the eyes 
of a monkey and then destroyed the angular convolution of the same 
side; this caused total blindness in the remaining eye of the opposite 
side, as was completely verified by numerous experiments. The ani- 
mal, however, showed some signs of returning vision the next day, but 
died as the result of exhaustion, the weather being extremely cold. In 







Fig. 16.— Scheme of the optic tracts and visual centres. A, the right and A', the left angular gyrus ; 
C, optic chiasm ; E, the right and E', the left eye ; n, the right and n', the left optic nerve ; O, the right 
and O', the left occipital lobe ; T, the right and T', the left optic tract ; the thin continuous line repre- 
sents the retinal relations of O ; the thick continuous line represents the retinal relations of O' ; the 
interrupted line indicates the retinal relations of A, and the dotted line the retinal relations of A' ; the 
relations of A and A' with the eye on the same side are indicated by finer interrupted and dotted lines 
respectively. (Ferrier.) 

another experiment Eerrier carefully examined the condition of vision 
in a monkey in which he had completely destroyed both angular gyres, 
and recorded the results as follows: 

" There was no ptosis, the ocular movements were normal, the con- 
junctival reflexes unimpaired, sensibility was intact everywhere, and 
the motor powers were perfect, but for four days at least the animal 
was evidently absolutely blind. When urged to move it ran against 
every obstacle in its path, paid no attention to threats, could not find its 
food, except by groping, and appeared insensible to light flashed in its 
eyes. On the fifth day there were evidences of returning vision. It 
did not knock its head against obstacles ; would not walk over the edge 
of the table ; showed signs of perception of light flashed in its eyes and 
occasionally seemed to wince when threatened. Vision gradually 



CORTICAL CENTRES 105 

improved, but continued very imperfect, especially for minute objects 
which it rarely, if ever, seized quite precisely; groping at them with 
the whole hand, and reaching short, or over, or to the side. It ap- 
peared to see objects held above, below, or to either side much better 
than those held in front of its eyes. Six weeks after the operation my 
colleague, Professor McHardy, examined the animal, which was very 
docile with me, testing every portion of the visual field by pieces of 
apple suspended by a delicate thread. It was concluded that vision 
was better in every part of the periphery than in the centre. Objects 
held directly before the eyes and at a little distance were apparently 
not clearly seen, and never laid hold of with precision. The condition 
remained unchanged for three months after the operation, similar tests 
being from time to time applied, and with the same result. I noted also 
that the animal, when examining any object, always held it at full 
arm's length from its eyes. The phenomena observable in the animal 
were such as would be best explained by impairment or loss of central 
vision." 

A few cases have been recorded by Demange, Sharkey, and others 
which appear to indicate that a destructive lesion of the angular gyre 
of one side in man will cause loss or great impairment of vision of the 
opposite eye, with contraction of the visual field of the same side. In 
a case recorded by Demange, 43 complete loss of sight on the side oppo- 
site to the lesion was present, as well as hemianesthesia and loss of 
hearing, taste, and smell. The lesion in this case was a large subcorti- 
cal area of softening extending beneath the convexity of a large part 
of the right hemisphere. The mesal aspect of the hemicerebrum and 
the internal capsule were nowhere implicated. 

One of the cases frequently referred to and especially used by 
Gowers and Ferrier in support of the view that crossed amblyopia is 
due to lesion of the angular convolution, is that of Sharkey. 44 In this 
case there were left hemiplegia, more marked in the arm and leg than 
in the face, dimness of vision, impairment of hearing on the left, loss 
of taste and of common sensibility on the left half of the tongue, and 
also loss of sensibility in the entire left half of the body. The paralysis 
of the leg largely, and the anaesthesia entirely, disappeared. The entire 
right hemisphere was much reduced in size, and, according to the 
report, the convolutions supplied by the right Sylvian artery were 
nearly all necrosed and atrophied. The crossed amblyopia was one of 



43 Demange, Revue Do Med., May, 1883, p. 301 

44 Sharkey, Med.-Chir. Trans., London, 1884, p. 205 



106 THE EYE AND NERVOUS SYSTEM 

the most interesting of the clinical phenomena present. The blindness 
in the left eye was almost complete at first. Improvement in sight, as 
in the other special senses, took place in about four weeks, and in six 
weeks and a half vision was completely restored. 

A number of clinical cases without necropsy, in which crossed am- 
blyopia has been present, have been recorded by Terrier and others. In 
these cases a series of other unilateral phenomena have, as a rule, been 
associated with the dimness of sight, these being usually such as hemi- 
anopsia, hemianesthesia, partial or complete impairment of other 
special senses, astereognosis, and hemiparesis. Doubtless in not a few 
instances the jDartial amblyopia is present in organic cases with uni- 
lateral symptoms, but is overlooked. In one of the cases reported by 
Ferrier, the patient was unaware of the impairment of vision in the 
eye on the side of her other symptoms until her attention was called 
to the fact by special examination. Not infrequently one eye with 
perfect or good vision serves so well the purposes of the individual that 
impairment of sight in the other does not attract attention until some 
incident or investigation reveals its existence. In these clinical cases 
of crossed amblyopia, marked impairment of the color sense is often 
present. In one case green was the only color recognized. The visual 
fields, determined with such sight as is left, are greatly contracted. 

Gowers gives brief notes of a case which is instructive in connec- 
tion with this question of crossed amblyopia. The patient was a 
woman fifty years old whose right eye had been removed many years 
before. She had marked concentric restriction of the field of the left 
eye accompanied by amblyopia. Accompanying symptoms were left 
hemianesthesia, dulness of the senses other than sight, and persistent 
headache. The patient was not an hysteric, but syphilis was probable. 
The patient's amblyopia and other symptoms improved under the use 
of iodide. 

While these cases undoubtedly show the possibility of the occur- 
rence of a form of crossed amblyopia from lesion of the angulo-occipital 
region, it is not to be inferred that they prove that this region is the 
only area of representation of clear or macular vision. In the cases 
of Demange and of Sharkey, the lesions were such as to involve the 
angulo-occipital subcortex, and may have severed the connections be- 
tween a lower and higher macular centre or even the macular bundle 
from the pregeniculum to the primary macular centre. The now some- 
what numerous cases of precortical and cortical word-blindness, with 
or without letter-blindness, have not shown, or at least not in the rec- 
ords given, that clear visual perception was lost. Such cases sometimes 



COKTICAL CENTRES 107 

have normal acuity of vision for objects other than words and letters 
both near and remote, as in one case studied by me recently. If the 
angulo-occipital cortex is the sole area of clear or macular vision in 
man, acuity of vision for all purposes would suffer in such cases, instead 
of the defect being confined to vision for words and letters. The an- 
gular gyre is simply a part of the higher visual or psycho-visual area, 
the particular part in which is located vision for the highest and last 
form of visual symbolization — that of language. 

The experiments of Ferrier and Yeo and Lannegrace might be 
interpreted as indicating that even in the monkeys experimented upon 
there is a higher area of clear or macular vision located in the position 
asserted by Ferrier. These animals, from destruction of the angular 
convolution on the one side, had transient loss of vision in the opposite 
eye, and even when the same region of the opposite side was destroyed, 
the total loss of vision which resulted was partially recovered from if 
the animal survived sufficiently long. This does not necessarily show 
that the cortical area destroyed was the only area for clear or macular 
vision, but, taken together, the experiments might be regarded as show- 
ing that lower as well as higher macular cortical centres are present 
in the monkey as well as in man. In the man these centres, as the 
result of evolution, become cortical areas of representation of designs 
and symbols; in the monkey they are probably the seat of memorial 
images of objects of small size and particular form. The fact must 
also not be lost sight of that in the experiments upon the monkey, the 
macular bundles, geniculo-cortical and intercortical, or both, must 
have been injured or severed. I believe in an angulo-occipital macular 
.area in the human being, but it is one which is concerned not only with 
clear or macular vision, but with vision whose purpose is the recogni- 
tion of words, letters and numbers, and probably also geometric and 
other designs. The proper place for the further discussion of the symp- 
toms which result from destruction of this higher macular area of the 
cortex is where word-blindness and other forms of visual aphasia are 
later considered. 

It is not improbable that there is for the macular field, as for the 
peripheral fields, a half representation, both primary or lower and sec- 
ondary or higher, in each hemisphere ; in other words, that a strictly 
limited lesion of the calcarine cortex on the one hand and of the angular 
region on the other may cause blindness in half of the macular field 
of the corresponding sides. At the same time it is probable that this 
half macular representation is not so strictly defined by a vertical 
dividing line as is the peripheral retinal representation. Putting it in 



108 THE EYE AND NERVOUS SYSTEM 

another way, the macular centre of one side is more or less representa- 
tive of the macular fields of both sides. 

Elsewhere 45 I have recorded two interesting cases which seem to 
indicate that a very limited lesion of the cortical area for word-vision 
or of the tract leading to it from the lower macular centre, might 
cause a loss of vision for one-half of a word, what might be termed 
hemianopsic amblyopia. One-half of the central field is obscured 
or lost, the peripheral field being maintained, or it may be partially 
obscured. The patient is half blind for words. A word of more than 
four or five letters will only be half seen, or at least will be partially 
cut off, unless the patient turns his eyes so that the unimpaired half 
of the central field can take in different parts of the word in succession. 

It is probable not only that lower and higher cortical macular cen- 
tres exist, but also lower and higher peripheral representation in the 
cortex; it is probable, moreover, that the lower macular region is con- 
nected with the higher macular region by its own bundle, and the lower 
peripheral portion of the fields with the non-macular portion of the 
higher visual field by its own fibre tracts. It follows that certain symp- 
toms will result from disease of the lower macular centre or of the tracts 
going from it to the higher centre of the same side and to that of the 
other side ; it also follows that certain affections will result from lesion 
of the fibres connecting the lower and higher peripheral cortical visual 
fields. The macula is not only the organ of acute vision, but of the 
highest form of psychic vision. The retinal periphery and its lower 
and higher cortical areas of representation are concerned with percep- 
tions and apperceptions of a lower order. 

With the facts just given before us, in order to apply the views 
advanced to all clinical macular phenomena, let us glance for a moment 
at the different forms of focal disease, peripheral and central, giving 
macular symptoms. Macular or central amblyopia will of course be 
produced by the destruction of the macula itself, but lesions of intra- 
ocular origin come almost exclusively under the attention of the ophthal- 
mologist. I would, however, direct attention to the fact that the macula 
is the part of the retina which is chiefly concerned with the recogni- 
tion of words, letters and numbers. Complete destruction of a large 
portion of the macula produces what might be termed, for convenience, 
a peripheral alexia and visual agraphia, while at the same time very 
considerable peripheral vision for form remains; in other words, the 

43 Mills, The Nervous System and its Diseases, — Diseases of the Brain and 
Cranial Nerves, p. 760. J. B. Lippincott Company, Philadelphia. 



CORTICAL CENTRES 109 

patient suffering from a large degree of macular destruction will still 
be able to orient himself with his retinal periphery and also recognize 
natural objects, individuals, buildings, and, to a considerable extent, 
designs and structures of various sorts. 

Central amblyopia results from a small infiltrating lesion, a nodule 
of sclerosis or a focus of hemorrhage, for instance, which may by rare 
chance be so situated as to destroy the macular bundle alone in the optic 
nerve and tracts in any part of its course. Clinical experience has 
shown that this bundle is especially liable to be affected by certain 
toxic agents. 

As the fibres of the macular bundle are undoubtedly distributed to 
the pregeniculum, complete destruction of this body or of a special 
portion of it, should cause central amblyopia. This central amblyopia 
would be of the crossed variety, and if only one external geniculate body 
were destroyed it would be transient, or would at least be recovered from 
in part. It is not yet positively proved that the pregeniculum is the 
basal centre for the macular bundle alone, as is held by Knies 46 and 
others. Some facts point otherwise, as for instance the case reported 
by Henschen, which seemed to show that hemianopsia in the lower quad- 
rant may be caused by a lesion of the dorsal part of the pregeniculum. 
In any case, if the periphery of the retina is represented in the pre- 
geniculum the macula is also represented in this ganglion, and if the 
terminus of the macular bundle is involved in a completely destructive 
lesion, crossed amblyopia, as above indicated, would result. Partial 
lesions of the basal macular centre, on one side or both, cause scotomata 
which may be transient or disregarded if the lesion is unilateral, or per- 
sist in consciousness if bilateral geniculate lesions are present. Para- 
central scotomata, peripheral vision, and central vision in the main 
remaining intact, should result from lesions which only partially de- 
stroy the macular geniculate centre. Such a clinical case has been 
recorded by Knies. In this case diminution of the hemianopsic pupil- 
lary response was present, that is, light thrown on one side of the retina 
caused a prompt iritic response, while on the other side the reflex was 
sluggish. A form of dyslexia apparently due to the impairment of 
macular vision was present in this case, which would seem to bear out 
the view that the macula is the part of the retina chiefly concerned with 
word- and letter-vision. 

Leaving the basal centres, a lesion of the macular bundle in the optic 



40 Knies. The Relation of the Eye and its Diseases to the Diseases of the Body. 
New York. 1S94. 



110 THE EYE AND NERVOUS SYSTEM 

radiations should cause some degree of central amblyopia ; this would 
he transient and more or less partial according to the degree of involve- 
ment of the fibres constituting this bundle. Here, as in the case of the 
pregeniculum and the optic tract, small destructive lesions would be 
represented symptomatically by scotomata. Bilateral lesions of the oc- 
cipital macular bundle would cause permanent amblyopia. In all the 
cases which have been considered, the amblyopia would be of the crossed 
variety from unilateral lesion, and in all probability close examination 
would show some impairment of vision with contraction of the field on 
the side of the lesion. 

In our consideration of this subject we have now reached the macu- 
lar centre in the primary or lower cortical visual field. Presuming 
the existence of this centre, have we any clinical evidence in support 
of this view ? As has already been stated, its existence is predicated 
by the occurrence of hemianopsia less a small central portion of the 
half field near the fixation point from a cuneo-calcarine lesion, and it 
is made still more certain by those cases of double hemianopsia in which 
a small central area of acute vision remains. Cases which suggest a 
lower macular representation are those in which outer portions of the 
retinal half field retain vision, while the rest of the half field to the 
dividing line is obscured. Here again also the question of paracentral 
and central scotomata becomes of interest. A lesion partially destruc- 
tive of the cortical macular centre of one side should cause a scotoma, 
paracentral or central according to its position. This would doubt- 
less be transient or disregarded, but might be elicited by special inves- 
tigation. The cortical macular centre of the opposite side would serve 
the purposes of vision, especially if the nasal macular centres were intact. 
Bilateral disease of the cortical centres for the macula causes persistent 
scotomata, or central amblyopia if the lesions completely destroy the cen- 
tres on both sides. Higher central vision, that for letters and words, 
for instance, would necessarily go with the partial or complete destruc- 
tion of the primary visual centres, although, as has been indicated, the 
reverse is not the case. Word-blindness or letter-blindness may be 
present without destruction of central visual acuity for other purposes 
than the recognition of language symbols, but a lesion or lesions destroy- 
ing primary central perception would necessarily disturb or destroy cen- 
tral apperception. 

The manner in which hemianopsia from cortical or cortico-subcor- 
tical lesion sometimes clears up or rather diminishes, indicates how a 
central or paracentral scotoma might be produced from a lesion limited 
to the lower cortical macular centre of one side. Hemianopsia in some 



CORTICAL CENTRES 111 

cases diminishes with comparative uniformity from centre towards 
periphery, the region of visual perception gradually expanding. In 
other cases it may clear in a lower or upper quadrant, or in an irregular 
area in any portion of the hemianopsic field. In still others, however, 
an irregular scotoma may remain as the field clears peripherally. In a 
case of this kind a portion of the cortical macular field has been com- 
pletely destroyed, the same being true of that portion of the peripheral 
cortical area farthest removed from the macular centre. An inter- 
vening area of both central and peripheral representation has escaped 
complete destruction. 

As already indicated, I believe that a distinct bundle passes from 
both primary cortical macular centres to the higher centre for clear vis- 
ion on each side. The tract from the lower centre of the same side prob- 
ably passes by way of the occipito-parietal subcortex, while the tract 
from the other side crosses through the splenium of the callosum to con- 
join with it, the conjoined tracts going to the angular convolution. A 
lesion of this conjoined tract or lesions of the tracts from both primary 
centres before they unite would, of course, cause disturbance or loss of 
the functions of the higher centre for which they are destined ; in other 
words, as will be shown more fully later, if the precortical lesions were 
left-sided, a variety of word-blindness would occur. Doubtless also close 
examination in such cases would show, at least at first, some impairment 
of vision in the eye of the side opposite to the lesion, this being quickly 
recovered from because both eyes are represented in each macular centre. 

Recently, in conjunction with Dr. C. D. Camp 47 I have published 
the history of a case of unusual interest in connection with the subject 
of crossed amblyopia, and also of that of visual hallucinations the result 
of organic disease of the cerebral cortex and subcortex. The patient, a 
woman sixty-three years old, had suffered with impairment of vision in 
both eyes for nearly two years before coming under observation. This 
dimness of vision was attributed to glaucoma for which a double iridec- 
tomy was performed some months before coming under observation. 
After this operation her vision was fairly good. I am indebted to Dr. 
Gr. C. Harlan, who performed the operation for the relief of the glau- 
coma, for notes regarding the state of the patient's vision and eyes at 
this time. Sight was reduced to 20/40 in the right eye and 20/50 in 
the left and the disc of each eye was cupped to the extent of about 1.50 
D. The fields were practically normal with the exception of a slight 
peripheral cut in the right eye. 



47 Mills and Camp, Trans, of the American Medico-Psychological Association, vol. 
xii. 1905; also Amer. Jour, of Insanity, 1005. 



112 THE EYE AND NERVOUS SYSTEM 

I saw this patient first in September, 1904. A few days before my 
visit she had a cerebral seizure in which the right eye became totally 
blind and vision in the left eye was greatly impaired. She had also lost 
the power of standing and was abnormally excitable. 

She was brought to Philadelphia where she was attended until her 
death by Dr. Harlan and the writer. A careful examination of her eyes 
by Dr. Harlan showed that in the right eye she had scarcely more than 
light perception. The cupped nerve had filled up to the level of the 
retina. In the left eye vision was reduced to 5/40 ; the field was periph- 
erally contracted to 40 degrees on the nasal and to 20 degrees on the 
temporal side. The disc was cupped but the fundus was otherwise nor- 
mal. Later this patient developed acute delirious mania with vivid and 
varying hallucinations of sight, which are described in some detail in 
the paper to which reference has been made. 

The patient died about three months after her sudden attack of loss 
of vision. 

Macroscopic examination showed the arteries at the base of the brain 
highly sclerotic and both pregeniculse atrophied. 

In sections from the calcarine fissure from each side were seen areas 
of intense congestion, with the formation of numerous very fine new 
capillaries. They were mostly in the second and third layers of the cor- 
tex, and the pyramidal cells in their vicinity appeared degenerated on 
examination by the Nissl method. Sections from the cortex of the lat- 
eral surface of each occipital lobe, near the occipital pole, showed areas 
not involving the whole section of a similar vascular appearance, as in 
the calcarine fissure, and probably a part of the same pathological pro- 
cess. The nerve cells in the location of these areas were of the pyra- 
midal variety, but they had lost their normal shape and structure com- 
pletely. The white substance beneath these areas appeared degenerated 
by the Weigert-hasmatoxylin method, and full of small holes as though 
nerve fibres had dropped out. 

The left optic nerve was about one-third of the normal size of an 
optic nerve, and surrounded by a greatly thickened sheath. There was 
much connective tissue overgrowth between the nerve bundles, which 
were small and degenerated. The chiasm appeared to be degenerated 
by the Weigert-hsematoxylin method, but there was no round cell infiltra- 
tion about it. 

Whether the complete blindness in one eye and the serious loss of 
vision in the other were to be regarded as constituting a genuine case 
of crossed amblyopia such as has been reported by Eerrier and Gowers, 
and attributed to lesion of the angulo-occipital region or of the macular 



COKTICAL CENTKES 113 

bundle, may perhaps be regarded as doubtful. If due to the lesions un- 
doubtedly present in the calcarine cortex, this" crossed amblyopia is of 
much interest in connection with the question of the existence of a sepa- 
rate macular representation in the primary or lower cortical visual cen- 
tres of the calcarine region and in the higher angulo-occipital visual 
areas. Dr. Harlan agreed with me that the final amblyopia in this case 
could not be attributed to the preceding glaucoma, at least not to the 
peripheral conditions which were present. With the exception of the 
cupped nerves, the fundus of each eye was normal and the filling up of 
the nerve cup seemed to indicate the occurrence of some intracranial 
lesion. The attacks of sudden loss of vision which occur in chronic glau- 
coma may, in some instances, be due to intracranial lesions affecting the 
cerebral, and especially the macular, bundles or centres. It is possible 
of course that the atrophy of the left optic nerve and tract may have ac- 
counted for the great loss of acuity of vision and contraction of the vis- 
ual field in the left eye, but in any case the sudden amblyopia in the 
right eye was doubtless due to a sudden vascular lesion in the left hemi- 
sphere. 

The phenomena of hysterical unilateral amblyopia would seem, when 
considered carefully, to confirm the theories held regarding crossed am- 
blyopia as the result of a unilateral organic lesion. In unilateral am- 
blyopia of hysterical origin, which is usually but not invariably asso- 
ciated with unilateral impairment of other senses, as of touch, pain, 
temperature, hearing, smell and taste, the vision is greatly impaired on 
the affected side. Examination shows in addition concentric contrac- 
tion of the field on this side. At the same time, some dimness of vision 
and less contraction of the field may be present on the other side. The 
same is true with regard to color phenomena. Dyschromatopsia may 
be marked on the affected side, and affected to a less and perhaps a slight 
degree on the other. These phenomena are the counterpart of what has 
occurred in the recorded cases of crossed amblyopia from organic lesion, 
the differences, if any, being in degree as to the impairment of vision, 
and also in the fact that the hysterical affection is usually transient, 
while the other is permanent or at least much more persistent. 

Whatever theory may be accepted in explanation of the hysterical 
amblyopia, it is clear that the affection is in some way dependent upon 
interference with the functions of the visual centres of one hemicere- 
brum. Whether this is in the nature of an angiospasm of cortical vessels, 
as once held, or of a temporary impairment of nutrition of the cortical 
cells, the fact remains that the symptoms point to a unilateral cerebral 
disturbance. It is significant that in hysterical cases hemianopsia in 
8 



114 THE EYE AND NERVOUS SYSTEM 

any of its varieties is not present, but that the loss of vision is central or 
general for the side affected. This is similar to what occurs in the ex- 
perimental lesions produced by destruction experiments upon the mon- 
key, or in cases like those of Sharkey, Demange and others, in which in 
the human subject cortical or subcortical substance has been destroyed. 
All the cases, organic or functional in man and organic in the lower 
animals, show the possibility of the occurrence of a crossed affection of 
vision distinct from that which gives anopsias or hemianopsias for light, 
color and form. It is true that the recorded cases of crossed amblyopia 
are few in number, but as Ferrier suggests, and as one or two of his clin- 
ical cases seem to demonstrate, the amblyopia in some instances may be 
overlooked. Whenever a patient presents such symptoms as hemianes- 
thesia and astereognosis, the case should be examined for unilateral dis- 
turbance of vision and of the other senses. The investigator should not 
be satisfied with demonstrating that hemianopsia is not present, or even 
that some central vision is retained in both eyes, but should examine not 
only for the presence of amblyopia, but for the degree of the retention 
or loss of central vision in both eyes. 

The visual auras of epilepsy, like the spectra of migraine, have been 
considered clinically in the present volume by Dr. Lloyd. I shall only 
direct brief attention to the well-known facts which indicate that these 
auras and spectra are usually the evidences of cortical disease, indicating 
either discharge or inhibition or both of the cortical centres for sight, 
primary and secondary. These auras take the form of either simple sen- 
sations of luminosity, of colors, or of persons or objects or of groupings 
of these. 

In many instances the primary visual phenomenon is a light sensa- 
tion, as a flash or a ball of fire. In other cases the aura is a sensa- 
tion of color which often assumes some definite form, as that of a ball or 
a star. It is significant that the colors seen are those which are recog- 
nized by most authorities as primary colors, as red, blue, green or yel- 
low, or, in rare cases, purple or violet. In the recorded cases red and 
blue have been most frequently present, sometimes alone, sometimes in 
combination. One or the other has been almost invariably present. 
Auras representing visual ideas are comparatively infrequent, but have 
nevertheless been observed in considerable number. These in particu- 
lar cases have taken the appearance of an individual in a certain colored 
dress, as for instance, a little old woman in brown. The primary sensa- 
tion of light or the sensation of color in a few cases, has preceded in the 
same seizure that of some objects representing a visual idea ; and Hugh- 
lings Jackson has explained this sequence of events by the discharge of 



VISUAL APHASIAS 115 

primary visual centres followed by that of secondary or higher visual 
centres. The latter, however, may take place alone. 

Some of the most carefully recorded observations regarding the spec- 
tra of migraine show that these, whatever form they may take, tend to 
occupy only the peripheral portion of the retinal field. The central field 
is nearly always free from these spectra, and the zigzag or other lines 
diminish in length as they approach it ; but this central field is subject 
on the other hand to inhibition, becoming obscured or absolutely dark- 
ened. 

It is only necessary in this connection to emphasize that the clinical 
phenomena thus recorded clearly indicate in the vast majority of cases 
that the cause of the phenomena is the discharge of centres and areas of 
the cortex which either act independently or in more or less frequent as- 
sociation. Inhibition of cortical visual centres is indicated by the dimi- 
nution or loss of sight which occurs as the sequence of luminous and 
chromatic sensations. 

THE VISUAL PORTION OF THE CEREBRAL ZONE 
OF SPEECH AND VISUAL APHASIAS. 

Under the general designation visual aphasia are included all those 
forms of interference with speech and with writing which result from 
lesion or disturbance of the visual centres concerned with language and 
the tract associating these centres with lower, co-ordinate, and higher cer- 
ebral centres and areas. While our concern is chiefly with the visual 
aphasias and the visual subdivision of the general zone of language in the 
cerebrum, a glance at this zone in its entirety forms a proper introduc- 
tion to the discussion of those parts of it which relate to speech as it is 
understood through sight. 4S 

The cerebral zone of speech includes an extensive cortical region, 
probably not less than one-third of the cortical expanse of the lateral as- 
pect of the left hemicerebrum, as shown in the shaded portion of Fig. 
17. While for descriptive purposes it is best to designate it as a cortical 
area, it must be understood that both the cortex and the sub-cortex of 
the entire region enter into its formation. 

Within this great zone are several important centres or sub-areas con- 
cerned with particular attributes of language. In Fig. 17 these centres 
are WH, a centre for w T ord-hearing, the auditory portion of the zone of 



48 A sketch of the cerebral zone of speech given in the text and the diagram 
illustrating it are taken from the article by me published in the American Journal 
of the Medical Sciences, September, 1904, the text of this article having been 
adapted to the present chapter. 



116 



THE EYE AKD NEKVOUS SYSTEM 



language ; at S and S a great centre for speech, often called motor ; at 
WS, LS, NS, a visual subarea concerned with the representation of 
words, letters and numbers ; at W a graphic or writing centre, usually 
spoken of as the motorgraphic centre ; and at N a naming centre in 
which it is believed by those who hold to its existence that names are cor- 
related with the concrete concepts of the objects named. 

Briefly stated, the auditive centre for speech is situated at the junc- 
tion of the first and second temporal gyres about opposite the point 
where the horizontal branch of the Sylvian fissure turns upward pos- 
teriorly; the dynamic or motor speech centre occupies the hinder por- 




Fig. 17.— Cerebral zone of language. W, writing centre ; S, S, speech centre (language arrangement) 
for emission ; WH, word-hearing or auditory centre ; WS, word-seeing ; LS, letter-seeing ; NS, number- 
seeing ; N, naming-centre ; PS, PS, PS, higher psychic region ; WM, centre for movements of upper 
extremity concerned in writing ; SM, centre for movements concerned in speaking ; OS, OS, OS, area 
of object-seeing. 

tion of the subfrontal or third frontal convolution, and, according to the 
view of the writer, the insula or at least its anterior half; the visual 
centres immediately concerned with speech are located in the angular 
gyre; the site of the graphic or writing centre is the caudal third of 
the second frontal convolution, and the naming centre is probably situ- 
ated in the third temporal convolution. 

Connecting these centres with each other, and also with other por- 
tions of the brain, are subcortical tracts composed of the processes of the 
cell bodies which are located in the cortex and of their fibrillary con- 
stituents. These associating tracts are of a complicated kind, although 
their number and arrangement are capable of analysis if sufficient time 



VISUAL APHASIAS 117 

and study are given to the matter. Only one or two points regarding 
them need here be emphasized, — namely, that the different centres are 
connected each with the other by a system of to-and-fro association, so 
that the impulses from special areas probably pass in both directions. 

Surrounding the zone of language on all sides are centres and areas 
which have important relations with the function of speech. These are 
of at least three kinds, and an understanding of them may be reached 
by a study of Fig. 17. On the sensory or receptive side of the brain are 
areas or centres concerned with such functions as object-seeing and ob- 
ject-hearing. Objects which are not recognized cannot of course be 
named, and these centres for object recognition therefore are neces- 
sarily contributors to the sensory concept side of the zone of language. 
They are represented in Fig. 17 at the points marked OS. On the mo- 
tor or emissive side of the cerebral speech zone are centres at SM and 
WM which are concerned with the execution of the work prepared for 
them by the speech and writing centres. They are, in other words, the 
centres of representation of movements of articulation and enunciation, 
and of the movements of the hand and upper extremity generally. At 
PS in the prefrontal portion of the brain is a region where the highest 
psychical functions are especially represented. It is evident that in using 
language, attention, volition, and reasoning play a part, although this 
part may vary within wide limits. This higher psychical region, there- 
fore, has its particular relation to the zone of language, and especially 
to its dynamic side. 

Let us now concentrate our attention on that subdivision of the cer- 
ebral zone of speech which is especially concerned with visual language, 
its lesions causing the different varieties of visual aphasia. As indi- 
cated in what has just been said, at WS, LS, and NS in Fig. 17 is situ- 
ated the cortical centre for one of the forms of higher visualization, the 
centre or region in which are stored the visual images of words, letters 
and numbers. This portion of the cortex is, as has already been shown, 
one of the subdivisions of the concrete memory field. It is connected on 
the one hand with the lower or primary cortical areas for vision in both 
hemispheres ; and on the other, with the areas for speech, writing and 
movements in the cortical zone cephalad of the central fissure, while at 
the same time it has numerous associating tracts with centres — contrib- 
utory or co-ordinate in function — like those for object-seeing, for stere- 
ognostic perception, for hearing, taste and smell. It is also connected 
with the special centre or area (N"), whose chief function is the associa- 
tion of names with objects, concepts of which are obtained through the 
special senses. If the reader has a clear idea of the location and connec- 



118 THE EYE AKD NERVOUS SYSTEM 

tions of this higher portion of the visual area, which is usually spoken 
of as the area for word-seeing, he can readily comprehend the different 
varieties of aphasia which result when this centre, its outlets or inlets, 
or its associated or associating structures are diseased. 

The most important varieties of visual aphasia and allied disorders 
are: (1) mind- or object-blindness; (2) word-blindness; (3) letter- 
blindness; (4) number-blindness; (5) alexia, paralexia and dyslexia; 
(6) visual agraphia; paragraphia and dysgraphia; (7) optic aphasia 
or word-dumbness. Some objection might be made to including mind- 
blindness in the category of visual aphasias, and such objection is doubt- 
less valid, but as a matter of convenience and with some scientific basis, 
I have thought it well to include mind-blindness under the general head 
of visual aphasias. The patient who is unable through sight to recog- 
nize a familiar person or object, or to recall the visual memories of such 
person or object, cannot of course name that of which he is not cogni- 
zant, and hence is aphasic in this indirect sense. Psychic blindness is, 
however, a disorder distinct in most respects from visual aphasia, and it 
will be therefore understood that it is only included here because of con- 
venience, and because it is so frequently associated with the true cerebral 
sensory disorders of speech. 

MIND-BLINDNESS. 

Mind-blindness is that symptom or condition in which the indi- 
vidual, because of cerebral lesion or disturbance, is unable to recognize 
by sight once familiar objects. Old visual memories of the kind re- 
quired for recognition of particular objects are no longer recalled. 

In order that mind-blindness shall result, it is necessary that the 
disease shall be situated either in the cerebral cortex or in the tracts 
which associate certain parts of the cortex. Such disease being present, 
all other parts of the intracranial apparatus may be normal, and yet 
the patient will exhibit this symptom. 

Numerous synonyms of mind-blindness are used and serve to a cer- 
tain extent to confuse the subject. The most important of these are 
soul-blindness, psychic blindness, and object-blindness. Visual apraxia, 
to which brief reference will presently be made, is still another syno- 
nym, and Wyllie has suggested the term visual pragmatagnosia, from 
the Greek -Kpay;xa y an object ; and ayvuxTia, a not knowing. It is pos- 
sible to have lost the power of recognizing an object at sight and yet 
be able to recall the appearance of the object; or both the ability to 
recognize the object at sight and to recall its image may be lost together. 
Wyllie has suggested that the term visual pragmatamnesia be applied 






MIKD-BLINDKESS 119 

to the loss of the power of calling up in memory the appearance of an 
object. A little consideration will show that in the difference between 
visual pragmatagnosia and visual pragmatamnesia we have a distinction 
similar to that which is drawn between cortical word-blindness and pure 
word-blindness, or between true color-blindness from cerebral disease 
and cerebral color amnesia. When the patient has lost the power of 
recognizing an object by sight, but retains the ability of recalling it as 
a visual memory, the cortex concerned with the function has escaped 
disease, but the tracts associating it with the lower or primary centres 
for vision are destroyed; just as in cortical word-blindness the visual 
memories of words cannot be recalled, whereas in the precortical, or 
so-called pure word-blindness, the visual memories can be recalled, but 
the individual is not able to recognize words through vision. 

The term mind-blindness or object-blindness might in a general sense 
be made to cover word-blindness, letter-blindness and number-blindness ; 
indeed these are but varieties of a general object-blindness. As our 
knowledge of cerebral function and cerebral disease increases, it is prob- 
able that we shall be able to classify mind-blindness into a number of 
sub-types. The uncivilized, like the higher forms of lower animal life, 
have keen powers of recognizing natural objects and the living beings 
around them. As evolution advances, added powers of recognition are 
developed. Doubtless some of these, like the ability to recognize colors 
with their various shades and tones, artificial designs and geometric 
forms have their special regions of representation in the general area of 
object seeing; and disease limited to such special areas or centres gives 
rise to special types of mind-blindness. The area concerned with the 
recognition of words and letters is not the only one developed in the 
educated or trained human being, but it and another sub-area of large 
extent concerned with the recognition of persons, places and objects, nat- 
ural and artificial, are all that have so far been differentiated. It is for 
the reasons here indicated that I have subdivided the area for object see- 
ing as shown in Fig. 3 and Fig. 5. While mind-blindness in its known 
forms is often associated with word- and letter-blindness and other forms 
of visual impairment or loss, this is not always the case ; and in a few 
recorded instances the inability to recognize faces or persons has been 
greater than that for objects of various sorts. A woman in advanced 
years under my care, who as the result of one or more cerebral seizures, 
suffered from word-blindness, mind-blindness and other often associated 
phenomena, was entirely unable to recognize her own sons by sight, al- 
though she was largely able to recognize her surroundings and orient 
herself with regard to them. 



120 THE EYE AND NEKVOUS SYSTEM 

The term apraxia introduced by Kussmaul, is not exactly synony- 
mous with mind-blindness or soul-blindness, but includes this affection 
and more. As denned by Kussmaul, apraxia is the loss of the memory 
of the uses of things and the understanding of the signs by which things 
are expressed. Usually these are matters of visual memory and the signs 
usually become known to us through sight, hence mind-blindness and 
apraxia in such cases signify the same thing; but the patient may be 
apraxic through other senses than sight ; if, for instance, he is unable to 
recall through hearing the nature of the object or objects which emit the 
sound, he is mind- or soul-deaf, — that is, he is apraxic through the sense 
of hearing. The rustling of leaves or the sound of a waterfall, or the 
notes of a bird may fail to recall the objects producing the sound, just as 
in the word-deaf, the sounds of words convey no meaning and in the 
music-deaf, musical tones are unrecognized as such. Tactile apraxia is 
exhibited when the patient is unable to recognize the nature and use of 
an object through touch. In this section we are concerned with apraxia 
only so far as it relates to sight. 

Burr 49 reports the case of a woman sixty years old who, while sit- 
ting at the table, suddenly lost vision. This patient, when examined by 
Burr, was mind-blind, and in addition she failed to recognize objects 
with the hands. She could not, for instance, tell what a watch was when 
placed in her hands, and she could not feed herself because of her in- 
ability to recognize a spoon, knife and fork. She seemed to recognize 
the hand of another person when it was grasped and also a few other 
objects. Here the apraxia was through touch and perhaps stereognostic 
perception as well as sight. Weir Mitchell 50 had recorded a similar 
case in 1892. 

What has just been said regarding apraxia refers to sensory apraxia, 
— that is, to the disorder due to disease on the sensory or receptive side of 
the brain. Cases are on record, however, which seem to show that a 
motor as well as a visual or other form of sensory apraxia may be exhib- 
ited, and it is worth while to briefly refer to these cases as they belong to 
the matter in hand in a diagnostic way because it may sometimes become 
necessary to differentiate motor apraxia from visual or other type of the 
sensory disorder. Pick, 51 for example, has recorded a remarkable case 
which he believes to be one of motor apraxia. A man sixty-two years 
old had for three years before coming under observation, peculiar at- 
tacks. He would suddenly find himself unable to speak the words which 

w Burr, Jour, of Nerv. and Ment. Disease, May, 1897. 

50 Mitchell, Medical News, vol. lxii. Oct. 1892. 

51 Pick, Neurol. Centralbl., vol. xxi. Nov. 1, 1902. 



MIND-BLINDNESS 121 

he wished to utter or to do anything desired. He conld not read or 
write, and everything seemed to swim before his eyes. He had neither 
paralysis nor loss of hearing, and in three days he was again normal. 
Similar attacks occurred about once in two weeks, and were associated 
with slight tonic masseter spasm. On one occasion he saw a mist and 
fiery rings. 

One of the most remarkable cases of apraxia ever recorded has been 
reported by Liepmann. 52 In this case the disorder was unilateral ; the 
patient was neither hemianopsic nor mind-blind. The tactile and pain 
senses were much diminished in the right half of the body, especially in 
the right limbs. The power of localization and the sense of position and 
of movement of the limbs seemed to be absent. The only motor paraly- 
sis was in the lower branch of the left facial nerve. This patient 
had motor aphasia and could only say a few words, these being al- 
ways the same; in other words, they were the recurring utterances 
of Hughlings Jackson. He understood what was said to him, could 
read, and obeyed simple commands when they were written, provided 
his movements were not performed with the right limbs alone. The 
movements of the head, including those of the face and tongue, were 
apraxic on both sides. When he was asked to make any movement with 
his right hand, such as touching the nose with the finger, his attempts 
were unsuccessful ; but with the left hand the command was promptly 
obeyed. When he was tickled in the right ear he made no movement 
of defense; but with the left hand he tried to remove any object that 
was placed in his left ear. He was unable to remove with his right hand 
a needle stuck in his right thigh. When asked to pick up with his 
right hand one of several objects placed before him, he was unable to 
comply with the request. It is probable that he understood what was 
asked of him, because after he had failed to pick up with the right hand 
the desired article, he was able to pick it up with the left hand when 
simply told to use this hand, without the name of the article being men- 
tioned again. With the right hand he was completely agraphic; but 
with the left he was able to do mirror writing, although the letters were 
very irregular. His gait was normal. Occasionally he performed an in- 
tentional movement correctly. 

In this case the patient was supposed to be suffering from aphasia 
with dementia, a fact of medicolegal as well as of neurological inter- 
est. Liepmann believed the lesion existed in the third frontal gyrus and 

52 Liepmann, Monatschrift fiir Psychiatrie und Neurologic, July, August, and 
September, 1900, vol. viii. This case is cited by Spiller, with comments, in Pro- 
gressive Medicine, 1900-1901. 



122 THE EYE AND NERVOUS SYSTEM 

insula, without implicating to any extent the motor area, or the inferior 
or superior parietal convolutions. It is difficult, however, to believe that 
the lesion was so placed, or at least so limited, as I have seen and re- 
ported a case of destructive lesion of the left third frontal and insula, 
in which no such unilateral apraxia was present although the case pre- 
sented typical aphasia. 

The discovery of the symptom known as mind-blindness was due to 
the physiologist Munk, who had observed it as the result of several extir- 
pation experiments on dogs. He found in some cases, when he removed 
a portion of the occipital lobes, the dog, while still retaining the power to 
see objects, and to avoid them through the medium of sight, was evi- 
dently unable to recognize the meaning and use of such objects. The dog 
from which a portion of the occipital lobe had been removed, for exam- 
ple, no longer recognized his master, other dogs with which he had been 
long familiar, or the food which was placed before him, — except, in the 
last case, through the sense of smell. He gave to this symptom the name 
of seelenblindheit (soul-blindness or mind-blindness). He believed that 
this symptom was due to lesion of the region for visual memories, the 
visual memory field, the visual portion of the concrete memory field or 
concrete concept area of the writer. Elechsig holds practically to the 
same view, believing that not only mind-blindness but mind-deafness 
are due to lesions of portions of this great association area. 

The truth is that all symptoms which result from impairment or 
disturbance of concrete concepts or concrete memories are to be sought 
for in lesions of centres located in the great concrete concept area, or in 
the tracts going to or coming from the centres of this area, or associating 
them together. Mind-blindness results, or may result, from lesion of the 
visual cortex or subcortex of this area ; mind-deafness, from the audi- 
tory portion of this area ; astereognosis, from lesions of that portion of 
this area devoted to the muscular sense and stereognostic perception ; 
tactile amnesia, from lesions of the centres and tracts concerned with 
tactile recognition ; music-deafness, from lesions of the centres and 
tracts related to the function of intonation; and so on throughout the 
entire list of concrete memories and their centres and tracts. 

Munk's views regarding the extent of cortex sufficient to cause 
mind-blindness do not appeal to the writer, as they have not to others, as 
correct, — this for theoretical reasons and because of clinicopathologi- 
cal observation. Munk expressed the opinion that extirpation of the 
circular area of the cortex so small as fifteen millimetres in diameter 
and two millimetres in thickness would cause in the animal the phe- 
nomena which he designated as seelenblindheit. The term seelen- 



MIKD-BLIKDNESS 123 

blindheit was used by Munk in contradistinction to rindenblindheit or 
cortical blindness, in which the animal becomes totally blind to all im- 
pressions ; it is unable, in other words, to see any objects presented to 
it as well as to recall the image of one formerly seen. Observations on 
man show that, whatever may be the case with dogs, it is necessary 
that a somewhat larger area of the cortex on both sides shall be destroyed 
than that indicated by Munk; and yet he was not as far wrong as 
Goltz and some of his most caustic critics would have us believe. Cases 
are now on record, some of which will be presently given, in which com- 
paratively limited lesions of the visual cortex of both hemispheres have 
given rise to mind-blindness. It is not, however, correct to regard the 
regions, destruction of which causes mind-blindness, as those for clear 
or central vision ; while, as has been stated, the centre for the macula 
is the centre for clear vision and for the recognition of minute objects 
and objects with special forms, such as those for words, letters, numbers 
and geometrical figures, the cortical areas of peripheral representa- 
tion, like the peripheral retina itself, are capable of recognizing many 
objects. The truth is, as I have already stated, that mind-blindness 
itself needs division and has a considerable sub-divisional cortical rep- 
resentation. The individual may be mind-blind for persons, or places, 
or figures, words or letters, or any of these or any combinations of these, 
the powers of recognizing the others remaining. 

One of the most important of the early cases of mind-blindness is 
that recorded by Lissauer 53 in great detail and with a discussion by him 
of the pathology and symptomatology of the affection and the methods 
of studying it. The patient was a man eighty years old, who for three 
years before coming under observation had had attacks of dizziness 
which were often so violent that he fell. For about a year he had been 
forgetful ; he often could not recall, for instance, the ages, names and 
number of his children. After a special attack he complained that he 
could not see as well as before. He bumped against doors and other 
objects and could not go around alone. He mistook parts of his dress 
and all sorts of objects ; he did not recognize his own daughter. His 
mind was clear for familiar business matters. The attacks of mind- 
blindness were transient. 

On examination, while he showed signs of old age, he was not in 
any way demented. While he could speak intelligently and easily he 
was unable to recognize everything. By touch and hearing he at once 
recognized everything. His sight, except for his mind-blindness, was 

53 Lissauer, Archiv fur Psycliiatrie und Nervenlieilkunde, vol. xxi. 1SS9-90. 



124 THE EYE AND JSTEKVOUS SYSTEM 

good. He drew objects or designs but afterwards he could not recognize 
them. He perceived but could not apperceive. With the exception of 
the mind-blindness, nothing else of importance could be made out. His 
hearing was dull, but he showed no cerebral affection of hearing or of 
speech. He was not paralyzed and had no impairment or disturbance 
of sensation. His ocular movements and reflexes were good. 

The patient during six months after he first came under observation 
made some improvement, and then failed to make any further progress. 
At times during this period considerable vacillations were observed in 
the amount and degree of his mind-blindness. He would recognize 
objects at one time while he would fail to do so at another. Even after 
he had made considerable improvement he would occasionally show a 
degree of inability to recognize objects as bad as he had at any time. 
The case was not followed until death but is nevertheless one of much 
value because of the careful manner in which it was investigated and 
described. 

In a case reported by F. Miiller, 54 the left occipital lobe was almost 
destroyed by a tumor and softening, and the posterior part of the cal- 
losum was implicated in the softening, and the associating tracts con- 
necting the right occipital lobe with the left hemisphere and its speech 
centres were interrupted. In this way Muller explains the optic apha- 
sia and alexia which were present. Mind-blindness was also present in 
this case. 

Muller mentions that mind-blindness is often associated with optic 
aphasia and asymbolia, and he gives the reference to a number of cases. 
Even more commonly it is associated with alexia, and he refers to sev- 
eral examples. In a number of cases right hemianopsia existed. Mul- 
ler says that in all cases of pronounced mind-blindness with satisfactory 
necropsy (1892) disease of the occipital lobe was found, or of the parts 
adjoining, as in two cases in which the parietal lobe was affected. Of 
twenty cases, both hemispheres were diseased in fourteen ; and of these 
both occipital lobes were affected in twelve ; in one, the right occipital 
lobe and the left frontal lobe were affected ; and in one the left occipital 
and the right parietal lobes were affected. In six cases the lesion was 
unilateral, and of these the left hemisphere was diseased in four cases 
(in two the left occipital and in two the left parietal). In two cases 
the right occipital lobe alone was diseased. 

Wernicke, Wilbrand, Bruns, Stolting and others believe that the 
occipital lobes are of equal value in function, especially in the recogni- 

54 Muller, Arch, fur Psych., 1892, vol. xxiv. p. 856. 



MIND-BLINDNESS 125 

tion of objects, and that mind-blindness can exist only when both occip- 
ital lobes are diseased. Miiller thinks it has been shown satisfac- 
torily that mind-blindness may be caused by unilateral lesion, as the 
examination of the brain must be microscopic and not merely macro- 
scopic. It seems to him more probable that lesions of the outer portion 
of the occipital lobes, especially on the left side, cause mind-blindness. 
The destruction of the tracts in the callosum connecting the occipital 
lobes or one occipital lobe with other parts of the opposite hemisphere 
is of much importance in the causation of mind-blindness. 

Liepmann 55 has reported a case of practically uncomplicated mind- 
blindness in a music teacher, aged fifty years, who had an apoplectic 
attack, September, 1901. After this he did everything in a reverse or 
foolish manner as, for instance, washing in his soup. He did not recog- 
nize large objects, nor his own reflection in the mirror, but recognized 
persons by the voice. He did not know his own wife by sight; color 
perception was much disturbed. A small defect was present in the 
upper right quadrant of the visual field; vision was two-thirds. He 
could imitate large movements fairly well. Well-known objects were 
often wrongly named. Recognition through touch was slightly dis- 
turbed, but through hearing was normal. Motor aphasia was absent, as 
was also sensory aphasia, unless his wrongly naming objects might be 
so considered. 

The patient died in 1904, two years after the case was first recorded 
Dy Liepmann. A necropsy showed most interesting results. Lesions 
were found on both sides of the brain, on the left in the lower occipital 
convolutions, and on the right in the angular and second occipital convo- 
lutions. In the right hemicerebrum the area of softening was on the 
convexity, and extended from the angular gyre through the gray and 
white matter of the second occipital gyre. 

A case reported by Serieux 56 is of value notwithstanding the com- 
plication of object-blindness with other symptoms like word-blindness. 
This case, which was published in 1891, is cited by Wyllie. The pa- 
tient was a woman sixty-two years old, who in 1888 had a temporary 
paralytic attack with lasting visual disturbances, and in 1890 had a sec- 
ond seizure with epileptiform spasms, hallucinations of hearing, and 
delirium. When examined six months later, she was word-blind, 
agraphic, word-deaf, and paraphasic. She also showed marked mind- 
blindness with intact general intelligence. She was unable to recognize 



Liepmann, Allgemeine Zeitschrift fur Psychiatrie, Berlin, vol. lix, 1902. 
Serieux, Compt. Rend. Soc. de Biol., December, 1891. 



126 THE EYE AND XERVOUS SYSTEM 

her nearest relations, said that people around her seemed to be wearing 
masks, that her daughter's face appeared " flat as a skate." The object- 
blindness was most marked with regard to faces, although exhibited in 
less degree with reference to other things. She could recognize some ob- 
jects, but not others ; a comb, for instance, was mistaken for a pen 
holder. She died of pneumonia a few months after the observations of 
Serieux were made. At the autopsy, lesions were found in both hemis- 
pheres. In the left hemisphere were present patches of softening 
in the supramarginal (subparietal) gyre, and in the caudal extremity 
of the first temporal gyre ; in the right were large areas of softening in- 
volving the angular and supramarginal gyres, and also the caudal ex- 
tremities of the first and second temporal. 

So far as is known to the writer, the few other cases of mind-blind- 
ness which are recorded are not of equal value with those of Liepmann 
and of Serieux, but all point to the existence of centres for object recog- 
nition by sight in the temporo-occipital convolution from the second to 
the fourth or fifth. 

Two cases have fallen under my observation, of transient or recur- 
ring mind-blindness. In both of these cases operation was performed 
for the removal of a brain lesion, presumed to be a tumor, although in 
one case at least the result did not confirm this opinion. In this case 
the patient presented in the right half of the body disorders of sensa- 
tion, stereognosis and co-ordination. He was partially word-deaf, word- 
blind, and letter-blind, and late in the case showed marked hemiparesis. 
The operation was performed with the view of exposing the brain at 
the posterior extremity of the first temporal convolution. A large patch 
of necrotic tissue, having somewhat the shape of a pyramid, with its 
base towards the occipital pole of the brain, was uncovered. The lesion 
may have extended further than was revealed by the operation. It not 
improbably included the caudal extremities of the first and second tem- 
poral convolutions, and possibly more. 

The operation was performed four years prior to the writing of this 
article, and the patient is still living and in fair health, but is hemi- 
plegic, partially hemiansesthetic and aphasic. He suffered at times 
from curious attacks which can properly be described as periods of mind- 
blindness or object-blindness. Recently, I re-examined this patient. 
I found that his hemiplegic condition had gradually become worse, the 
paralysis having visibly increased, especially during the last year. He 
showed some slight improvement in the ability to name objects looked 
at, also in being able to name many words. He could not, however, 
form phrases or sentences. He seems still to be partially word-deaf,. 



WORD-BLIXDXESS: ALLIED DISORDERS 127 

being thrown into confusion in trying to understand much that was 
said to him. He continued largely word-blind, but recognized an occa- 
sional word, writing being understood rather better than printed matter. 

His wife, who accompanied him, stated that, beginning about two 
months after the operation, the patient had at irregular intervals spells 
of temporary mind-blindness or object-blindness. In these, which would 
last from a few minutes to one or two hours, while he would seem to 
know his wife, he was apparently unable to recognize others. In these 
attacks also he appeared unable to recognize objects or his surroundings ; 
he seemed dazed, would look around as if wondering who the persons 
and what the objects around him were, repeating almost constantly one 
word, " What, what." He could not understand anything that was said 
to him at the time of these spells, and became greatly confused and irri- 
tated. His wife said that he remembered these spells after they passed 
off, and would say " Awful, awful," as if in great dread of them. Ex- 
amination showed that he understood the use of most objects, such as the 
telephone, a pen, ink, shears, and paper. An effort was made to examine 
him for color-blindness ; he could not recognize red and blue, but seemed 
to know green and yellow; but when the examination was continued, 
he became confused, and was unable to recognize any colors. 

In the second case referred to, in which operation exposing the occip- 
ital lobe was performed, and portions of the cortex removed or con- 
tused, corresponding to the parts of the first and second occipital con- 
volutions, the man suffered not only from optic aphasia, but tem- 
porarily from object-blindness. At first, he did not at times recognize 
persons and things. The impairment was only partial. 

Briefly stated, the pathological cause of mind-blindness is, in the 
first place, a lesion or lesions of the visual portion of the concrete mem- 
ory field. Usually the disease is on both sides of the brain, but transient 
forms of the affection, and perhaps, in exceptional instances, the per- 
sisting disorder may be caused by a destructive lesion of the left optic 
memory field. In the second place, lesions of the tracts which connect 
the higher visual field with the lower or primary visual centres may 
cause this symptom. When the affection is due to a lesion of the asso- 
ciation fibres it is, if unilateral, so situated as to destroy the tracts which 
connect the primary or lower visual centres on both sides with the higher 
visual area on one side. 

WORD-BLINDNESS AND ALLIED DISORDERS. 

In several places in the present chapter reference has been made to 
the fact that in the angular convolution of the left cerebral hemisphere 



128 THE EYE AND KEKVOUS SYSTEM 

and its immediate neighborhood is situated a centre for the storage of 
the visual images of words. It is not infrequently spoken of as the 
centre for word-seeing. It is the last and most important development 
of the higher visual area, the visual portion of the concrete memory field. 
A destructive lesion of the cortex of this region or of the tracts entering 
it from the primary or lower visual centre will cause the symptom to 
which Kussmaul gave the name, word-blindness. One of the best defini- 
tions of word-blindness is that of Bateman, who describes it as a form 
of verbal amnesia in which the patient has lost the memory of the con- 
ventional meaning of graphic symbols. He sees these symbols, he knows 
that they differ from their environments, but he cannot translate them 
mentally into words. They are to him as if they had never had any 
significance. 

The two chief varieties of word-blindness now usually described are 
known respectively as cortical word-blindness and pure word-blindness. 
By cortical word-blindness is meant that variety of this form of aphasia 
which is due to lesion limited to the cortex ; while the term pure word- 
blindness was applied by Dejerine to the form which is dependent upon 
a lesion of the tracts which enter the cortical centre for word-seeing from 
the primary visual centres. The term pure word-blindness like pure 
word-deafness does not seem to be a happy one, but is perhaps better 
retained, as it has become fixed in the literature of the subject. It is 
objectionable in the sense that a number of similar designations are of 
doubtful clearness, — as, for instance, Munk's soul-blindness (seelen- 
blindheit), which is used as a contrasting term with his cortical blind- 
ness (rindenblindheit) : one designation is founded upon the psycholog- 
ical idea, that of psychic blindness, and the other is anatomico-patho- 
logical in its origin, referring to the blindness caused by the destruction 
of cortical brain substance. 

In cortical word-blindness the patient is unable to comprehend words 
by sight and is also agraphic. Owing to the loss of the memory of visual 
images, he cannot recall these for the purposes of writing. In the pure 
word-blind patient, while he cannot comprehend words, either printed or 
written, by looking at them he may be able to write spontaneously or 
from dictation, as his cortical centre for the visual images of words and 
letters is not destroyed, but if his eyes be removed even for a moment 
from the written page he cannot recognize the word which he has just 
written. 

While we owe to Dejerine a clearer conception of the manner in 
which word-blindness usually originates, the observation of two forms 
of word-blindness, one in which the patient is agraphic, and the other 






WOKD-BLHSTDKESS: ALLIED D-ISOKDEKS 



129 



in which he retains the power of writing from dictation or spontane- 
ously, is not new. Westphal, who is cited by Kussmaul, many years ago 
recorded the case of an actor who was hemiplegic and incompletely 
aphasia Although he could speak with fluency, he could not recognize 
or understand some words. He was completely alexic, and while he 
could write from dictation, he immediately afterwards could not com- 
prehend the words he had written. 



Ano .Qyn 




Fig. IS.— Dejerine's diagTam showing the position of the lesion causing pure word-blindness. 

The exact view of Dejerine with regard to the manner in which pure 
word-blindness is caused can be best shown by a reference to the diagram- 
matic illustration, Fig. 18, which is reproduced by permission from 
Wyllie's work on the Disorders of Speech. 

"An examination of this diagram (Fig. 18) will enable the reader to 

understand quite easily the relation of the eyes to the primary visual 

centre in the occipital lobe. In the diagram, the left optic tract and its 

connections are, for the sake of distinctness, given in deep black. The 

9 



130 THE EYE AND NEKVOUS SYSTEM 

reader will, on looking at the diagram, at once understand how section 
of the left optic tract, BO, would produce homonymous hemianopsia in 
the opposite half of the field of vision. He will also see that the same 
effect would be produced by a lesion further back, either in the optic 
thalamus, in the radiations of Gratiolet, or in the primary cortical centre 
itself. But he will note that word-blindness will not, in any of these 
cases, be added to the hemianopsia, unless the lesion be situated at about 
x, and so cut across not only the fibres which connect the angular con- 
volution with the left occipital lobe, but also those which connect it with 
the right one." 

As the early cases illustrating the difference between cortical and 
precortical word-blindness are comparatively well known, I shall refer 
here only to a few cases somewhat recently reported. 

Dejerine and Thomas 57 report an additional case of a woman who 
developed right hemiplegia and hemiansesthesia with right hemianopsia 
and simultaneous loss of the power of writing eight years before her 
death. After a slight temporary paraphasia she regained the power of 
speaking; she understood all that was said to her; she was able to 
write, but immediately afterwards could not understand what she had 
written ; she was word-blind, with the exception that she could recognize 
an occasional letter. Two years after her first apoplectiform attack she 
had a second, and after this she became unable to write at all, either 
spontaneously or from dictation, her other symptoms remaining much 
the same. The lesion in this case was very large ; so large, indeed, as to 
interfere a little with reasoning on the basis of the findings. It was 
important with regard to the views of Dejerine as to the cortical word- 
blindness and pure word-blindness that the angular convolution escaped. 
A large focus of softening involved the third temporal convolution and 
the posterior ends of the first and third occipital, while ventromesially it 
destroyed the cuneus, the fusiform, lingual and subcallosal gyres, 
the hippocampus and cornu ammonis ; the pulvinar and internal genicu- 
late body were also destroyed. The patient died from recent hemor- 
rhage. 

One of the most recent cases bearing upon the subject of pure word- 
blindness is recorded by Hinshelwood, Macphail and Ferguson, 58 this 
report also containing several other facts of great interest to the student 
of aphasia. A man, fifty-three years of age, who was a teacher of French 
and German, suddenly found that -he had lost entirely the ability to read 



67 Dejerine and Thomas, Rev. Neurol., July 15, 1904, p. 645. 

68 Hinshelwood, Macphail, and Ferguson, Brit. Med. Journ., Nov. 12, 1904. 



WORD-BLIKDKESS: ALLIED DISORDERS 131 

words or letters. He could write correctly to dictation but could not, a 
short time afterwards, read what he had written. He had a right lateral 
homonymous hemianopsia. He was under observation for nine years 
until his death, no other symptoms developing. He was able, only to a 
very limited degree, to reacquire the visual memories of words. His 
death was due to a hemorrhage into the right half of the cerebellum as 
was shown by necropsy. The lesion causing the word-blindness, letter- 
blindness and hemianopsia was a necrotic cyst in the occipital lobe. The 
cortex destroyed was summarized by the recorder as a large, triangular 
area limited by the calcarine fissure internally and by the third tem- 
poral gyre externally, with the apex at the occipital lobe. The cor- 
tex of the cuneus with the exception of the posterior portion of the 
calcarine fissure was not destroyed ; the fibres passing to and from the 
cuneus were, however, involved as were also the fibres going from the 
cuneus to other portions of the occipital cortex, and also the fibres passing 
to the left angular gyre from both the left and right primary cor- 
tical visual centres. As the left angular gyre was intact, it will be 
seen that the case, as the authors assert, supports the views of Dejerine 
and of Serieux as to the distinction between precortical or pure word- 
blindness and cortical word-blindness. 

I have emphasized the views of Dejerine with regard to the distinc- 
tion between his pure word-blindness and cortical word-blindness, or 
what I would prefer to call precortical or qualified word-blindness, and 
cortical or absolute word-blindness, and also have given his hypothesis as 
to the position of the lesions in these two forms of visual aphasia illus- 
trating these facts by references to a few recorded cases. It must not 
be understood, hoivever, that the views of Dejerine necessarily explain 
all cases of his so-called pure word-blindness. Cases are on record which 
do not bear out the supposition that this form of visual aphasia is always 
dependent upon a precortical lesion. Several cases are now on record 
in which the so-called pure word-blindness or pure word-deafness or both, 
in the same case, were present, yet the lesion in whole or in part affected 
the cortical centres for word-seeing and word-hearing. Strohmayer 59 
has examined the cases of pure sensory aphasia accessible to him, six in 
all. He points out the important fact that in five of these cases the 
lesions were bilateral, implicating both the cortex and the subcortex of 
the temporal lobe. In only one case was the lesion unilateral, and here 
it was confined to the left temporal cortex. From a study of these cases 



50 Strohmayer, Deutsche Zeitschrift f . Nerrenheilkunde, vol. xxi. Nos. 5 and 6, 
p. 371. 



132 THE EYE AND NEKVOUS SYSTEM 

he argues that the lesions causing pure sensory aphasia are bilateral, 
although, as has been indicated, a few cases like those of Dejerine and 
Serieux, for instance, seem to make this doubtful. 

As has been shown in a preceding section of this chapter, according 
to the views of Lannegrace, Eerrier, Gowers and the writer, each angular 
gyre is to a certain extent a centre of representation for both macuke. 
If this be the case, it might be possible for the patient to be word-blind 
from lesion of the higher visual area of one hemisphere with or without 
a precortical lesion or a lesion in the opposite hemisphere and yet retain 
some power of writing spontaneously and from dictation with the help 
of the undiseased angular convolution. 

Hinshelwood 60 recently reported a case which seems to show that 
when an individual knows more than one language he may become word- 
blind to one language alone, or the word-blindness may vary in degree 
in the case of the different languages. He had a patient who became 
unable to read English, but could read Greek, and had partial word- 
blindness for Latin. On testing this man for Erench, a greater degree 
of word-blindness was manifested than in the reading of Latin. Even 
with English the word-blindness was not absolutely complete, and there 
was no letter-blindness. Hinshelwood believes that in the case of a per- 
son who is able to read several languages, the letter and word visual 
images of each language will be grouped together, forming thus a series 
of separate groups within the centre for word-seeing. If the whole centre 
is destroyed or its functional activity abolished, the individual will be 
word-blind to all languages ; if there is only a partial interference with 
the centre, then the word-blindness may be only partial and may be lim- 
ited to a single group of these visual changes, — i.e., to a single language. 
The same writer refers to another patient who was word-deaf to French, 
his native tongue, but could understand what was said to him in Eng- 
lish. The explanation offered is similar to that given for his case of 
word-blindness to languages; in other words, if in the word-hearing 
centre, the auditory images of different languages are arranged in sep- 
arate groups, in a partial lesion one group may be interfered with or 
destroyed, while the others are preserved. 

Usually in word-blindness as in any other form of sensory aphasia 
or of motor aphasia, the lesions determining the symptomatology are 
located in the left hemicerebrum ; the only exceptions to this presumably 
being those cases in which the cerebral affection occurs in left-handed 
persons. Of the few cases which have been seen to confirm this left- 

60 Hinshelwood, Lancet, Feb. 8, 1902, p. 358. 



WORD-BLIKDKESS: ALLIED DISORDERS 133 

sided localization the right-handedness of at least some of the patients 
affected with the aphasia has been in doubt. 

Recently in conjunction with Dr. T. H. Weisenburg, I have pub- 
lished the account of a case of marked sensorimotor aphasia associated 
with left hemiplegia and some other phenomena. 61 

The patient at the age of thirty-five had a severe apopletic stroke. 
He was seen by me a day or two after the seizure and then presented 
the usual phenomena of a severe cerebral hemorrhage. When he re- 
covered from the immediate effects of the attack, he was found to be 
completely hemiplegic on the left side and almost absolutely aphasic. 
He was seen by me only once or twice at times closely related to 
his apoplexy. I saw him, however, a year later when he regained 
the use of his left extremities so that he was able to walk about with 
the usual hemiplegic gait, and his aphasia had improved to a certain 
extent. 

About a year after his first seizure he had a convulsion, three or 
four the following year, and the third year still more. Dr. Harvey 
Gushing, of Johns Hopkins University, performed an osteoplastic opera- 
tion designed to completely cover the motor region of the right side, and 
to a less extent, the area posterior to it. After the operation, up to the 
time of his last coming under observation, which was about a year later, 
he had one or two spasmodic attacks. These attacks were mostly, if not 
altogether, left sided. 

Eour years after he was first seen by me in his acute apoplectic con- 
dition, this patient returned and remained under my care for about two 
months, during which time systemic efforts were made to improve his 
aphasia by training, Dr. Weisenburg being associated with me in this 
work. At this time a careful study was made of his cerebral symptoms. 
In the first place, he still had marked hemiplegia, the leg, arm and 
face being affected, although the last to only a slight extent ; the deep 
reflexes on the paralyzed side were exaggerated and a typical Babinski 
was present; on the entire left half of the body he was hypothetic to 
touch, pain, temperature and to vibration sensation (the sensation which 
results from the placing of a tuning fork on bony or other points) ; 
sensation of position was partially abolished in the left hand, in which 
he was also largely stereognostic ; the ophthalmoscope showed a normal 
fundus and partial left lateral homonymous hemianopsia. He was not 
word-deaf, or, if so, only to a very limited degree ; he understood what 
was said to him with perhaps rare exceptions. He could repeat any- 



61 Mills and Weisenburg, Medicine. 1905. 



134 THE EYE AKD KEKVOUS SYSTEM 

thing that was said to him, his articulation and enunciation being 
excellent. 

His most interesting aphasic phenomena were in the visual sphere. 
Immediately after his attack he had been undoubtedly word-blind and 
letter-blind. At the time when the careful examinations were made, his 
word-blindness and letter-blindness still remained in very large degree. 
He was not, however, object-blind, distinctly recognizing persons, and 
also the use and nature of objects around him. He knew a few letters 
as, a, b, d, o, s, which he had learned as the result of many months of 
teaching. Even the letters which he knew he could not pick out from 
the midst of the word in which the letters were present. As the result 
of prolonged teaching, he could only recognize a few words, and these 
were chiefly those with which he had been extremely familiar in his 
past life. He could, for instance, pick out the words apple and seed, he 
having been in a business in which such words were frequently used, 
both by mouth and by written signs. He could sign his own name or 
he could write his own name with the appended words "and wife;" 
if what he had written was presented to him later, however, he seemed to 
be utterly oblivious of its meaning. He had learned to write his own 
name with the added words " and wife" in order to register at hotels. 
He could do this as well with his eyes closed as open. He was an 
alexic and visual agraphic. 

Close investigation of this man's history seemed to show that he 
was born right-handed. He ate, wrote, and did by preference almost 
everything with his right hand. When he was four years old he broke 
his right arm, and during the time that he was unable to use it, he 
learned to do many things with his left hand. He would, for instance, 
play ball with his left hand by preference. This tendency had ap- 
parently arisen out of the conditions of the fractured right arm. 

He did not write with his left hand, and was not, in the ordinary 
sense of the term, ambidextrous. We can assume, therefore, that the 
patient was congenitally and by acquired habit a right-handed individ- 
ual. The lesion in this case was undoubtedly on the right side, and was 
either a subcortical hemorrhage involving the tracts going to the pre- 
central, postcentral, superior parietal and angular convolutions, and 
possibly the upper part of the first temporal convolution, or an embolus 
of the right middle cerebral artery. The optic radiations may, to some 
extent, also have been involved. 

The important features of this case were (1) that in a right-handed 
man with left hemiplegia aphasia largely of a sensory type appeared and 
this aphasia was persistent; (2) the aphasia consisted principally in 



WORD-BLINDNESS: ALLIED DISORDERS 135 

word- and letter-blindness, and this was due to a lesion in the right 
side of the brain. 

Instances of crossed aphasia, the term first applied by Byrom Bram- 
well, are very rare. By crossed aphasia is meant aphasia which is, so 
to speak, against the rule. As is well known, aphasia usually occurs 
from a lesion of the left hemisphere and in association with right-sided 
paralysis, but in crossed aphasia the symptom is due to a right-sided 
lesion in a patient who is right-handed, or a left-sided lesion in a person 
who is left-handed. 

Senator, 62 who recorded one of the most recent cases in 1904, was 
able to collect only eleven cases from literature, one or two of which 
are somewhat doubtful; and in all, with the exception of Senator's 
case and the one we now report, the hemiplegia was right-sided and 
occurred in left-handed individuals. 

The causes of crossed aphasia may be explained in several ways: 
( 1 ) , the lesion may involve the cortex of both sides ; this, however, is 
exceptional and was not present in any of the recorded cases ; (2), there 
may be no decussation of pyramidal fibres, the lesion being on the same 
side as the hemiplegia. This is exceedingly uncommon, having only 
been recorded in a single case; (3), the lesion causing aphasia in a 
right-handed individual with left hemiplegia may be on the right side, 
and in a left-handed person with right hemiplegia on the left side. 

It is not necessary to consider the first two hypotheses in the case 
just given, for a lesion of the right side undoubtedly caused the left 
hemiplegia and aphasia. It is difficult, however, to explain why the 
centre for word- and letter-seeing should be situated on the right side 
in a right-handed individual. 

The question, of course, arises as to what factors determine on which 
side of the brain the motor speech centres and the more specialized 
centres, as of word- and letter-seeing, will be localized. All things 
considered, in a child which has not as yet learned to write or read, 
heredity, congenital tendency and acquired habit determine whether the 
child shall be right- or left-handed. We can assume, therefore, that a 
child whose parents are right-handed and in whom there is no physical 
deformity to prevent the use of the right hand, will be right-handed, and 
that its leading speech centre (presuming that both sides of the cortex 
have to do with motor speech) will be located on the left side in Broca's 
convolution. The converse, of course, is true in left-handed persons. 

This, however, is by no means always the case. Weber 63 records 

6a Senator, Charite annalen, vol. xxviii. 1904. 
63 Weber, Centralbl. f. Physiol., xxiii. Nr. 12. 



136 THE EYE AKD KEKVOUS SYSTEM 

instances of children who had not as yet acquired the ability to write, 
in which a lesion on the right side would cause aphasia in right-handed 
persons, and vice versa. In other words, in children, Or for that matter, 
in adults, who had not as yet learned to write, Weber assumes that 
the motor speech centres are potential on both sides of the cortex. This 
is probably so, for it is improbable that the speech centres are equally 
divided between the two sides of the cortex, even in childhood. Here 
heredity, congenital tendency and acquired habit will probably make 
the child either right- or left-handed, and therefore right- or left- 
brained. 

The factor which determines on which side such highly specialized 
centres as those for word- and letter-seeing would be localized is probably 
the art of writing. This, no doubt, also determines on which side the 
leading motor speech centres will be. This was well shown in a patient 
of Bramwell. 64 A man of thirty-six, who was left-handed for every- 
thing except for writing, had right hemiplegia with persistent motor 
and. sensory aphasia, evidently from a lesion in the left hemisphere. 

A case of Oppenheim 65 also illustrated this point. A woman of 
fifty-nine, who, since she was seventeen, had been left-handed because of 
an injury to the right hand, developed complete motor and sensory 
aphasia, due to a tumor in the right cerebral hemisphere. 

In the patient whose case is just recorded, however, the man always 
had been right-handed and had always written with his right hand. At 
the age of four, as already stated, he had broken his right arm and had 
learned to do many things with his left hand. Even for those purposes 
for which he commonly used his left hand, he could and sometimes did 
employ the right. We must assume, therefore, that although he wrote 
with his right hand, and was born and continued right-handed, except 
as stated, his motor and sensory speech centres were situated in the right 
hemicerebrum. On inquiry it was learned that the patient's parents, 
grand-parents and collateral relatives were all right-handed. It must 
be assumed, therefore, that in exceptional cases, in spite of the usual 
laws of heredity and of acquired tendency, there is, for some unknown 
reason, an inclination toward the localization of the motor and sensory 
speech centres in that side of the brain on which they would not ordi- 
narily be expected. 

It is equally interesting to note that in the case discussed the aphasia 
is almost wholly confined to word- and letter-blindness. Koster 66 records 

64 Bramwell, The Lancet, June 3, 1899. 

65 Oppenheim, Archiv f. Psych., vol. xxi. 1890, p. 139. 

66 Koster, Abst. in Centralbl. f. Nervenheilkunde and Psychiatrie, February, 
1901, p. 117. 



WORD-BLIKDKESS: ALLIED DISORDERS 137 

a case with necropsy in which, with left hemiplegia in a left-handed 
person, there was Word-blindness, and in addition, left hemianopsia. 
At the necropsy extensive destruction of the right cerebral hemisphere 
was found. Koster believes that this was the first case reported with 
necropsy, of word-blindness due to lesion of the right hemicerebrum. 
This case resembles the case under consideration to a large extent, except 
that the patient was right-handed. 

The case here recorded is also instructive from another standpoint, 
for it teaches that not only in exceptional cases may the centre for 
letter- and word-seeing be situated on the right side, but also that a 
unilateral lesion may cause permanent loss of word- and letter-seeing, 
opposing the views held by Bastian and others that these functions have 
a bilateral representation. 

JofTroy 67 records the case of a right-handed man, aged 77 years, who 
was suddenly seized with mental agitation, after which he presented 
almost complete word-deafness and word-blindness. He could recognize 
some letters and could appreciate the meaning of certain words. Para- 
phasia, paragraphia, and sometimes jargon utterances were present. 
He could write his name with difficulty, but could not copy nor write 
from dictation. Death occurred from cerebral hemorrhage three months 
later. Examination of the brain showed that the left hemisphere was 
normal. The right hemisphere presented a patch of softening involving 
part of the posterior third of the first and of the second temporal gyres. 
Around this patch shrinking of the convolutions and adherence to the 
membranes were present, extending over the posterior two-thirds of the 
first and second temporal gyres and involving the whole angular convo- 
lution. 

JofTroy believed that this man was congenitally left-handed, but 
that he had become trained to be right-handed, and he offers this 
explanation for the situation of the speech centres upon the right side 
of the brain. ~No ancestral left-handedness is mentioned in the history. 

Letter-blindness (literal blindness, amnesia, liter arum) is a form 
of blindness due to cerebral cortical or subcortical disease in which the 
patient is unable to recognize letters, although the visual apparatus, 
from the periphery to the primary cortical visual centre, or cortical 
centre for optical perception, and this centre itself are intact. Letter- 
blindness may exist without word-blindness, and word-blindness without 
letter-blindness, although both are usually associated. Curious facts 
regarding blindness to letters from lesions of the cortical centre for 



67 JofTroy, Rev. Neurol.. Jan. 31. 1903. p. 112. 



138 THE EYE AND NEKVOITS SYSTEM 

higher visualization, or the entering tracts to this centre, are often 
observed. The patient may retain the ability to recognize one or two 
or several letters of the alphabet, but fail to comprehend all the rest. 
We believe that letters and words are represented in separate cell 
groups of the cortex, although these are adjacent. 

Alexia, the inability to read because of cerebral disease or dis- 
turbance, may be due to lesions variously situated. In its most complete 
and typical form it is dependent, as has already been shown, upon a 
lesion of the cortical centres for word-seeing and letter-seeing of the 
tracts which pass to these from the optic perception centres or primary 
cortical centres for sight in the calcarine cortex. Some degrees of alexia 
may, however, be present from a lesion of the centres for word-hearing, 
naming, or of Broca's centre, as well as of the tracts which connect these 
various centres. All of the centres in the zone of language take some 
part in the verification of the correctness of the varieties of speech 
spoken and written. 

It is held by some writers on aphasia that if the word-hearing 
centre is destroyed the patient will be unable to read. My own ex- 
perience, however, shows that this is not always the case, and Wyllie 
and others give cases in which word-deafness was present without alexia. 
In these cases reading is probably made possible by direct connections 
which exist between the visual centres for words and letters and Broca's 
centre. 

In so-called motor aphasia from lesion of the left third frontal and 
insula, alexia in some degree may be present, but it is certainly not 
absolute. In one of my cases which was under observation during a 
period of many years, I was able to demonstrate in various ways that 
the patient was able to read silently, although his reading may have 
been imperfect. When, however, he attempted to read aloud, he always 
repeated the same words. The visual images in this patient had the 
power to revive the auditory images, although perhaps not perfectly. 
Necropsy in this case revealed a destructive lesion confined to the left 
third frontal and insula. 

Paralexia, in which the patient transposes or substitutes words and 
syllables in reading, is caused by lesions variously situated in the zone 
of speech, as by partial lesions of the visual centres, by lesions of the 
auditory and motor speech centre and of the tracts connecting these cen- 
tres with the visual centres for language. Doubtless there is a silent 
paralexia, the patient in some cases transposing silently. 

Dyslexia, or fatigue in reading, the result of lesion or disturbance 
of the cerebrum, is sometimes observed. The patient may be able to 






WOBD-BLINDKESS: ALLIED DISORDEKS 139 

read correctly either script or text, to read aloud or to repeat what is 
read by others, but the effort soon becomes fatiguing, the reader tires 
and may be unable in a short time to go on with the reading. It is a 
■condition comparable to that which is observed in some cases of word- 
deafness which have partially recovered, and is sometimes associated 
with partial word-blindness. In one such case of word-deafness recently 
observed by me, the patient could listen attentively and understandingly 
to one person quietly talking to him for a short time, but if the conversa- 
tion was continued, and especially if it became more varied, his attention 
soon tired and before long he would entirely fail to appreciate what was 
said to him. If several persons were talking to him at the same time, he 
also became confused and unable to comprehend what was being said. 
Heading and writing of an imperfect sort could be continued for a short 
time, but even these became impossible after a few minutes. 

Dyslexia may be due to a partial lesion or a functional disturbance 
of almost any portion of the zone of speech. In its most typical forms 
the lesion or disturbance is usually in, or in close connection with, 
either the visual, the auditory, or the motor speech centre. It is some- 
times a premonitory symptom in apoplexy, doubtless indicating some 
ischemia in a region which subsequently becomes the seat of hemorrhage 
or occlusion. 

Agraphia and allied conditions, like dysgraphia and paragraphia, 
may be due, like alexia, dyslexia and paralexia, to lesions variously 
situated in the zone of speech, as in the centre for the visual images, 
that for auditory images, Broca's centre, and the so-called motor-graphic 
centre in the posterior part of the second frontal convolution. More 
or less complete agraphic disturbance may also be due to lesions of the 
tracts connecting these various centres. We are here, of course, chiefly 
concerned with visual agraphia, that form of impairment of reading 
which is dependent upon lesion of the visual centre for language or the 
tracts which lead to or from it. 

Stanley Barnes 68 has reported a case of graphic aphasia in asso- 
ciation with epilepsy in a case of cerebral tumor lasting nine years. 
The man's speech in this case was slow and paraphasic ; while he 
was able to name most articles, some with which he was less familiar 
than with others, he could not name. He, therefore, had partial optic 
aphasia or word-dumbness. He could not write as well as formerly ; 
this appeared to be due to the fact that he could not, except with 
difficulty, make out words on the right side of the line. He understood 

68 Barnes, Review of Xeurology and Psychiatry, vol. i. 1903, p. 531. 



140 THE EYE AND KEKVOUS SYSTEM 

what he read; he wrote slowly from dictation and very inaccurately, 
few words of more than three letters were spelled correctly ; the letters 
were badly formed. He copied from print to long-hand very slowly. 
In spontaneously writing he began well, but his efforts became worse and 
worse and in a half hour he could not write more than ten lines. 

The temporal field of the right eye was markedly contracted for 
all colors. In the left eye the nasal field was contracted about ten 
degrees. 

The patient grew progressively worse until his death. Many symp- 
toms and conditions not especially relating to the visual fields are 
omitted. 

Necropsy showed a tumor about the size of a small orange, occupying 
portions of the postcentral, superparietal and inferior parietal convolu- 
tions, extending into the white matter but not as far as the ventricles. 
The growth was a gliosarcoma. 

Admitting the existence of a cheiro-graphic or cheiro-kinsesthetic 
centre distinct from all other special centres, sensory or motor, Barnes 
argues from his case that the connections between the visual centres 
and both the graphic and motor speech centres, would be severely 
injured, this being accompanied by partial destruction of the visuo- 
auditory and cheiro-auditory commissures. It will be seen from Barnes' 
explanation that all forms of writing would be much impaired, while 
other aphasic symptoms were referable to partial injury of visuo- audi- 
tory commissures. His partial right homonymous hemianopsia was 
evidently due to lesion of the optic radiations. 

Barnes accompanies his paper with an interesting illustration which 
is reproduced in Fig. 19. 

Gulbenk 69 has described the case of a patient with a rare dis- 
turbance of the faculty of writing, which he terms dysantigrapJiia. A 
man, seventy years old, came into the hospital for difficulty with his 
speech and inferior facial paralysis, which had been present for about 
twenty days. Speech was slow and embarrassed, the words being badly 
pronounced but intelligible; syllables were not transposed and jargon- 
aphasia was absent. He had no paralysis of the limbs. 

The patient could write pages fluently and legibly to dictation ; but 
if asked to copy, he could only do one or two lines and then would begin 
to misspell and the writing would become illegible about the fourth line. 
If again dictated to, he could immediately write without fatigue. 

The aphasia, of which a trace was present on his admission, soon 

09 Gulbenk, Rev. Neurol., February 15, 1904. Abst. in Review of Neurology and 
Psychiatry, vol. ii. 1904, p. 322. 



WOKD-BLINTJXESS : ALLIED DISORDERS 



141 



developed more and more, becoming almost complete. At this stage 
writing was quite impossible. 

Gulbenk holds that this case shows that the truth lies between the 
view that our hand copies visual images stored up in the angular gyre, 
and the other that our hand translates directly into graphic signs the 
sounds furnished by internal speech. In writing we use at the same 
time visual images and these sounds. Admitting a graphic centre at 

RIGHT Cuneus LEFT 



- Angular gyrus 

Outer fibres of op. rad. 

Tumor 

Corpora geniculata 

\Vl.V^I^ I ^i su<>c h e i r Oo r aphic 
S" / V commissure 



Op. radiation 
cp. quad. a n . 



Blind part of visual 
field of R. eve 




- Asc. frontal eonvol. 



L. op. tract 



Retina of L. eye 



Visual field 



Fig. 19.— Diagram showing the manner in which writing and speech were impaired by destructive 
lesions involving the tracts between the visual and the cheiro-graphic and motor speech centres, and 
also involving the visuo-auditory and cheiro-auditory commissures. ' (Stanley Barnes.) 

the posterior extremity of the second frontal convolution, Gulbenk ex- 
plains the symptoms of the case by postulating a functional ischemia, 
leaving intact the connection of auditory (temporal lobe) with motor 
images, but interfering with the connection of visual images (angular 
gyre) with the same. This ischemia would affect the connecting fibres 
in their passage beneath the Rolandic convolutions, thus causing the 
paresis of the inferior facial. The author compares dysantigraphia with 
the dyslexia of Bruns, where the patient, after reading fluently a few 
words, finds it impossible to continue. 



142 THE EYE AND NERVOUS SYSTEM 

Numeral-blindness without either letter- or word-blindness is occa- 
sionally recorded; it would, perhaps, be more frequently observed if 
the attention of the examiner was directed to the subject. Hinshel- 
wood reports four cases of word-blindness in only one of which blind- 
ness for figures or numbers was present. In one of these cases the 
patient, a teacher of languages, became suddenly totally word-blind and 
letter-blind, and yet, when he was examined with the test-types, while 
he could not read a single word, he could at once recognize and repeat 
the numbers at the top of each paragraph. In this case, the lesion, a 
considerable area of softening, did not involve the angular convolution, 
although it did the white matter of the occipital lobe, including the tract 
from the primary to the higher visual centres and the optic radiations 
as well as some portions of the occipital cortex. 

Dejerine 70 has recorded a case of pure word-blindness without 
agraphia, due to a subcortical lesion, agraphia being added with the sub- 
sequent cortical lesion, in which case the patient was totally unable to 
read, and eventually, to write ; he retained the power of reading figures 
and could do mental calculations • as well as formerly. 

I have seen cases of word-blindness in which the power to recognize 
and use numbers in calculations was retained in whole or in part. In 
my diagrams, Eigs. 3 and 5, I have indicated a number-seeing centrs 
as distinct from that of word-seeing and letter-seeing, although adjacent 
to both. Hinshelwood accords with the view thus indicated, that ths 
groups of visual memories of letters and figures are deposited in dis- 
tinct but probably adjacent areas of the cerebral cortex. 

A few words might be said here about the treatment of visual 
aphasia, and especially of word- and letter-blindness. As many of these 
cases of word-blindness are the result of cerebral hemorrhage, Hinshel- 
wood urges the importance of complete cerebral rest, and above all, of 
preventing the patient from practising reading for at least a consider- 
able time after the attack. Many of these cases are the result of 
syphilis, and it is wise to put the patient on specific treatment if there 
is the slightest possibility of syphilis having been contracted. When 
actual destruction of the centre for word-seeing has occurred, re-educa- 
tion may call the corresponding centre of the right cerebral hemisphere 
into function, but re-education should not be attempted until all signs 
of acute brain disease have disappeared. 

The treatment of alexia, due to word- and letter-blindness is some- 



70 Dejerine, Comptes-Rendus des Seances de la Societe de Biologie, March, 1892. 
p. 64. 



WORD-BLIKDKESS : ALLIED DISORDERS 143 

times disheartening, but with perseverance much can be accomplished, 
at least with persons of intelligence and education. Of course, as audi- 
tory, and to some extent, other forms of aphasia, are commonly asso- 
ciated with the visual speech disorder, training for word-deafness, para- 
phasia and partial motor aphasia, may need to come hand-in-hand with 
that for word- or letter-blindness, but I shall speak here only or chiefly 
of direct visual training. 

The patient should be made to copy numbers, letters and words, 
separately and in various juxtapositions. Efforts should also be made, 
as time progresses, to have him write from dictation and spontaneously. 
After writing from dictation or copying, the patient should try to write 
the same numbers, letters, words or combinations by recalling them for 
this purpose. With regard to words, it is probably best to begin with 
nouns, following with verbs, and later with qualifying and conjoining 
words, which are best taught by presenting them in short sentences. 

Reference to the plan pursued in a recent case, the one recorded by 
Dr. Weisenburg and the writer, will perhaps give the best idea of the 
methods of training of most value for visual aphasia. 

Two methods are used at the present time in teaching children the 
recognition of words. In our Philadelphia public schools, the word sys- 
tem is used, — that is, the child is not taught to spell the word, but to 
recognize it en bloc. An apple is shown or is drawn on the board. The 
child is told that it is an apple, and after this is firmly impressed, the 
word apple is written on the board, and an attempt is made to fix the 
image of the word in the child's mind. It is claimed for this method 
that by it words, are acquired much faster. The child later learns of 
himself to apply the method of spelling, or, what is by no means 
unusual, never becomes a good speller. 

In the older method the child is first taught the alphabet, and then 
to spell the word letter by letter, and syllable by syllable. While this 
method is a slower one, the results are better as regards spelling. It 
is probable that a carefully considered combination of the two methods 
would work out the best results. 

Lowenfeld 71 records two cases of amnesic aphasia in which the 
patient, in one case, could not read short but could read long words ; in 
the other case, if the patient had difficulty in reading a word, instead of 
trying to spell it, he would attempt to pronounce it en bloc. This was 
the case with the patient treated by Dr. Weisenburg and the writer. 
He could at times recognize words, and often when he would try to 

71 Lowenfeld, Ueber zwei Fiille von amnestischer Aphasie. Deutsche Zeitschr. f. 
Nervenheilk., vol. ii. 1892. 



144 THE EYE AND NEKVOUS SYSTEM 

pronounce a word he would not attempt to either spell it or pronounce 
it in part, but as a whole. On being asked to recall a word with which 
he was familiar, his mental picture of the word was of its entirety; 
he could not recall its literal components nor spell it. In training him, 
both the word image and the letters method were used. In six weeks he 
was able to recognize nearly all the letters of the alphabet. Considering 
that he was only able to recognize about six letters when he came under 
our care, the improvement was marked. The letters were impressed in 
different ways. He was shown a letter, as p, made to pronounce it, to 
write it, to pick it out of other letters, and to try to picture it. At the 
same time, simple words were taught him in the same manner ; in other 
words, he was shown the word, made to pronounce it, to write it, to pick 
it out of other words, and try to recall its appearance mentally. The 
progress in the recognition of letters and words was about equal. 

AGRAMMATISM. 

One who has many opportunities to study aphasics soon becomes 
aware that in those cases in which language is not entirely lost or is 
confined to a few simple recurring utterances, like " Yes" and " No" or 
" La la," " Oh no," or some profane ejaculation like " Damn" or " Oh 
God," the chief disturbance of speech exhibits itself in a disorder of 
grammatical expression. The patient, with his mutilated speech, tends 
to recur to the forms of expression which are used by those who, like 
the Chinese, have a language which is undeveloped so far as gram- 
matical construction is concerned, or like the imbecile, the demented, 
or the victims of confusional insanity. Various parts of speech which 
are necessary to the full expression of the language ordinarily employed 
by the educated, or even by those who are largely uneducated, are 
omitted. The words used are transposed or emitted in grotesque con- 
fusion. The grammar of language has fallen into confusion or chaos. 
When this disorder of speech is the result of disease or defect, it is 
termed agrammatism or akataphasia, a subject which has received large 
attention from a few German and other writers, like Steinthal, Kuss- 
maul, and Pick. Kussmaul has devoted an interesting chapter to these 
syntactical disturbances of speech. He shows that a speaker, in order 
to express himself correctly in language, must have an unbroken flow 
of words, perfect grammatical diction, and a correct arrangement of 
words. One or two, or all, of these requirements may be impaired or 
lost. 

In the production of agrammatism or akataphasia it is evident that 
one must take into consideration not only the destruction of centres 



AGRAMMATISM 145 

and of tracts, but also the more or less general disturbance of the 
entire zone of speech as it has been defined and described in this chapter. 
According to the position and extent of a lesion, the grammatical or 
syntactical derangement may be of this or of that kind. When auditory 
speech centres or their incoming, outgoing, or associating tracts are the 
seat of disease, the grammatical as well as the philological disturbance 
of speech will be dominated by losses or impairments which show that 
auditory language has been the subject of the most severe and destructive 
assault; when visual centres and tracts are the seat of lesions, visual 
language will suffer the most in grammatical as in ordinary graphic 
expression; and in like manner, when the anatomical substrata of the 
sphere of language on its motor or emissive side has borne the brunt of 
disease or injury, the disarrangement will be most shown in the power 
of conception and of expression. In each of these cases grammatical 
construction will suffer to a greater or less extent. 

According to some authorities, these grammatical and syntactical 
disturbances of speech, or some of them, are due to the disease or defect 
in speech centres concerned with the co-ordination of languages ; accord- 
ing to others, the morbid phenomena are the result of the dissociation 
of the separate centres and areas concerned with language. Probably 
both views have some foundation in fact. 

As I have elsewhere pointed out, 72 the recognition of the part played 
by the impairment or loss of the power of grammatical construction or 
arrangement must receive special consideration in attempts at the 
restoration of speech by training. A citation from this paper on the 
Treatment of Aphasia may be of service in this connection. 

" It became a matter of interest to me in observing aphasics who 
were striving to recover their lost powers to note the particular parts 
of speech which were most obscured and most difficult to regain, and 
also the particular difficulties which attended their reacquirement or 
prevented this. A patient who has so nearly lost language that he has 
only one or two recurring utterances regains, by repetition and recall, 
word after word, especially nouns. Later a few verbs are acquired. 
Adjectives, adverbs, prepositions, and articles, the parts of speech whose 
uses are to modify, limit, or express relation, are so entirely lost to the 
patient as to cause more than anything else his difficulty in language. 
The same remark applies to conjunctions like and and but, and 
with a special force to the auxiliary verbs in all their moods and tenses. 



"Mills, The Treatment of Aphasia by Training, Journal of the American 
Medical Association, December 24, 1904. 

10 



146 THE EYE AND NEKVOUS SYSTEM 

The meaning and use of such words or combination of words as is and 
was and has been are in eclipse or lost to a varying extent. The impair- 
ment or loss of the faculty of constructing sentences or propositionizing 
in language is one of the most important if not the fundamental defect. 
While thought is at times expressed by a single word, the sentence is the 
usual unit of thought expression, and the aphasic patient, or at least 
some aphasic patients, find their chief difficulty in regaining those parts 
of speech which are concerned with qualifying and correlating. The 
grammar of language no longer exists for them. 

" Considerations of this kind led me to the use of primers and gram- 
mars in retraining of aphasics, and these with individuals previously 
reasonably educated are of great value. Patients should be taught the 
grammar as a child is instructed, — in other words, by teaching him the 
meaning of the different parts of speech and the exact methods of using 
them in phrases and sentences. The significance and value of the 
qualifying, relating and conjoining parts of speech should be enforced 
by numerous examples. When the aphasic, in his efforts to repeat after 
another or to respond to what another asks or indicates, uses incomplete 
sentences, the complete sentences should be given and explained. In 
every possible way the manner of using adjectives, prepositions, con- 
junctions, and auxiliaries should be impressed. The patient, with the 
book before him, should be taught to conjugate verbs, decline nouns and 
pronouns, compare adjectives, and in other ways go through the routine 
methods of studying language employed in the schools, these being 
modified by the particular requirements of the case. The dictionary 
can often be used as a valuable adjunct to the grammar, some patients 
taking a particular interest not only in reacquiring words in this way, 
but in thus learning their meaning and their uses as parts of speech." 

Close examination of the subject, especially in connection with the 
study of actual cases, shows that the faults in language arrangement 
from which these patients suffer are, in a strict sense, syntactical rather 
than grammatical, and the term akataphasia is probably more suitable 
than that of agrammatism. Syntax is " the due form and arrangement 
of words or members of sentences in their mutual relations according 
to established usage. Syntax includes the proper use of parts of speech 
and of forms in their combinations to make sentences and their proper 
arrangement or collocation." (Century Dictionary.) A consideration 
of the numerous examples furnished by partial aphasics, or by aphasics 
recovering from various forms of disturbance of speech due to cerebral 
disease, shows that their great impairment is of the faculty of construct- 
ing sentences, — that is, of arranging the words of a sentence in their 



AGRAMMATISM 147 

proper position and order. Grammar is a more general term, including 
syntax; but grammatical mistakes as they are commonly understood 
refer to the misuse of parts of speech which may be arranged in a 
sentence correctly from the syntactical point of view. It is a curious 
observation that aphasics, in the process of re-education, usually employ 
the parts of speech correctly or incorrectly according to their previous 
education, — that is, in so far as they regain their linguistic powers. 
Their sentences may be mutilated. Nouns may be present in consid- 
erable number, and they may employ some verbs and some qualifying 
and conjoining words; but the verbs used, if the patient is educated, 
are of the right mood and tense, and the substantives and pronouns are 
of the correct person and number. They do not use the plural for the 
singular or the present for the past. The uneducated, however, in 
proportion to their degree of education or their lack of it previous to 
the onset of their aphasia, make grammatical mistakes. As speech is re- 
acquired, the professor or Jimmie Fadden, whatever may be his syn- 
tactical reacquirements, reasserts himself grammatically each after his 
own kind. 

Pick, 73 in an article on agrammatism as the result of a focal lesion, 
gives several theories which have been held regarding this symptom. 
He believes that it is a so-called disorder due to lesion somewhere in 
the cerebral cortex, and holds that this lesion is situated in the left 
temporal lobe adjoining Broca's area. The case reported seems to prove 
this contention, for, although two areas of softening were present in 
the left hemicerebrum, one in the anterior part of the left supratemporal 
convolution and the other in the left third frontal convolution, Pick 
gives reasons for the opinion that the lesion in the anterior portion of 
the first temporal gyre was first in order of development and gave rise 
to agrammatism. Dejerine and his school, as is well known, hold to 
the sufficiency of Broca's area for all the purposes of motor speech and 
writing, and contend that agrammatism, like aphasia and agraphia, is 
only one of the varieties of a comprehensive motor aphasia. Bernheim 74 
has adopted the views of Dejerine, but, as shown by Pick, an important 
study of the cases reported by Bernheim will show that the lesion in 
these cases was in every case in the temporal lobe, or at least involved 
this lobe. 

Pick discusses the relations of grammatical expression to a cerebral 
substratum. Ziehen, for instance, holds that grammatical expression 



73 Pick, Zeitschrift f. Heilk., vol. xxiii. No. ii. 1902. 

74 Bernheim, De 1' Aphasie motrice, 1901, Ausdruck verleihen. 



148 THE EYE AND NERVOUS SYSTEM 

is not dependent upon the integrity of a co-ordinating speech centre, but 
is rather the result of associative combinations. Pick, however, holds 
to the existence of a language co-ordinating or grammatical centre which, 
as has been indicated, he places in the anterior part of the temporal 
lobe. 

VISUAL ANOMIA OR WORD-DUMBNESS. 

One of the most interesting of aphasic clinical phenomena is that 
which is illustrated by the inability of a patient having good vision for 
ordinary purposes, suffering from a lesion in the cerebral zone of lan- 
guage, to name by sight an object the use and nature of which he 
recognizes. For this symptom, which has long been observed by clini- 
cians, Freund, 75 who made the earliest elaborate study of the subject, 
suggested the name visual aphasia. Another term frequently used, 
especially by the Germans, is optic aphasia. Merkens 76 proposed to 
call the symptom word-dumbness, a term which has much descriptive 
appropriateness. It is, as Langdon 77 suggests, name memory-loss or 
anomia, or rather visual anomia. 

The preferable names are visual anomia or word-dumbness, although 
the term optic aphasia has probably had the largest use. The expression 
visual aphasia employed by Freund is objectionable, as it may lead to a 
misunderstanding. I prefer to use this name rather to designate the 
entire class of visual aphasic phenomena, and in that sense it has already 
been used in the present chapter as a general caption for all the visual 
affections of language which have an organic cerebral substratum. 

Occasionally word-dumbness is observed as an isolated form of 
anomia, — that is, it is only through sight that the inability to name 
known objects is exhibited; frequently the patient is unable to name 
objects which he recognizes through the sense of sight, and is also 
unable to denominate them through other senses, — as, for instance, that 
of touch or of hearing. The name memory-loss, in other words, may 
be not only for one but for several or all of the special senses. In one 
of my cases the names of objects could not be recalled either through 
sight or touch, but could by means of hearing. A gustatory or olfactory 
aphasia is possible, the patient being unable to name a rose, for example, 
through the sense of smell, — vision and the other senses being excluded, 
— or an apple through the sense of taste, with the same sensory ex- 

75 Freund, Archiv fiir Psychiatrie und Nervenkrankheiten, vol. xx. 1888, p. 404. 
79 Merkens, Deutsche Zeitschrift f. Chirurgie, vol. lx., p. 417, 1900. 
77 Langdon, The Aphasias and their Medico-Legal Relations. The Laning Print- 
ing Co., Norwalk, Ohio, 1898. 



VISUAL ANOMIA 149 

elusions. In still other cases the patient may be unable to name an 
object with the help of any senses, although he recognizes the nature 
and use of the object through all the senses. 

In order to place clearly before the reader the idea of general 
anomia or loss of naming power through any of the senses, a well- 
recorded case by Potts 78 is worthy of citation. A man who was left- 
handed for all acts except writing lost the power of naming objects, 
whether they were seen, heard, smelled, felt, or tasted. He compre- 
hended all that was said to him, knew the names of objects, and could 
converse fluently with the exception that he omitted the names of per- 
sons, places, and objects. He could not repeat names pronounced in his 
hearing, but he knew his own name and address and could write them ; 
but otherwise spontaneous writing was impossible. He copied fairly 
well but soon tired. He had had an apoplectic attack, the immediate 
symptoms of which, with the exception of the visual aphasia, had dis- 
appeared. In about two months he made such marked improvement 
that he was able to name after reflection most objects presented to him, 
and he was also able to write a letter. This man, although word-blind, 
could read numerals. Hemianopsia was not present. Potts argued 
that it was necessary to assume in this case either that the lesion cut a 
number of tracts leading respectively from the centre of visual, tactile, 
auditory, olfactory, and gustatory memories to the motor centre, or 
else that tracts from each of these centres converged to a common 
centre where the memories of names are stored up, to be called into 
consciousness by stimulation of one or all of the percipient centres, and 
that from this centre a tract passes to the motor speech centre and that 
this tract was destroyed. 

Personal experience with a number of cases with necropsies has 
demonstrated for the writer the fact that this symptom is present in 
lesions variously situated in the cerebral zone of speech, as the hinder 
part of the left third frontal convolution and the insula, in the third 
and fourth temporal convolutions, including both cortex and subcortex, 
at the junction of the posterior thirds of the first and second temporal 
convolutions, and again in these convolutions and the adjacent portions 
of the occipital lobe. Cases have been recorded which indicate that the 
symptom may arise from lesions situated in various portions of the 
occipital cortex or subcortex. Freund, after recording several cases of 
his own and reviewing the literature of the subject, explains the mech- 
anism of the occurrence of the symptom with the assistance of a diagram. 

78 Potts, Journal of the American Medical Association, May 4, 1901. 



150 THE EYE AKD JSTEKVOUS SYSTEM 

Bearing in mind the facts already presented regarding the centres for 
object-seeing and the various tracts and centres of the cerebral zone of 
speech, the production of the symptom from differently located focal 
lesions can readily be understood. 

It is first necessary to recall the writer's views regarding a naming 
centre. I have long held that between the sensory and motor cerebral 
centres for speech exists a concept area, in which probably is a naming 
centre. This has been located on theoretical grounds and as the result 
of a few clinicopathological observations in the midtemporal region. 
Presuming the existence of this centre, a centre concerned with the 
affixing of names to concrete ideas of objects recognized through the 
lower and higher centres for the special senses, it is evident that word- 
dumbness will occur as the result of destructive lesions variously 
situated. 

It has been shown that in the lateral occipito-parietal and occipito- 
temporal regions is situated an area concerned with object recognition ; 
also that destruction of the whole or a part of this area, in some cases 
on one side of the brain and in others on both sides of the brain, causes 
the symptom which is known as object-blindness or mind-blindness. Of 
course, if the patient cannot recognize objects he will not be able to 
name them. The symptom, therefore, may result from a lesion of the 
region or regions of object recognition. Word-, letter-, and number- 
blindness, for instance, are due to a destructive lesion of the angulo- 
occipital region or the tracts leading to it, and inability to name words, 
letters, or numbers will be present as a part of these forms of cortical 
and precortical visual aphasia. When objects other than words, letters, 
and numbers cannot be recognized, it is probable that a bilateral lesion 
is usually necessary for the production of not only mind-blindness, but 
of visual anomia. Tracts pass from the various centres for object seeing 
to the naming centre in the left temporal lobe. A lesion of these tracts 
in the left hemisphere will cause word-dumbness for those objects 
whose recognition is confined to this hemisphere. For objects recognized 
by both occipital lobes it will be necessary for the complete production 
of this symptom that the tracts passing from both hemispheres to the 
naming centre shall be destroyed. This may be brought about by a 
single lesion after the tracts unite, by bilateral lesions, one in the left 
and the other in the right occipital lobe, or one in the left hemisphere 
and the other in the splenium of the callosum, through which it is 
probable that the tract passes from the right to the left side. 

Lesion of the naming centre itself will also cause this symptom, and 
in this case it is probable that the word-dumbness will be not only for 



VISUAL ANOMIA 151 

objects recognized by sight, but for those recognized by the other senses, 
as the tracts from all the centres of the special senses probably converge 
or concentrate in the concept naming area. A case like that reported 
by Potts would, for instance, be most likely due to a lesion situated 
here. 

From the naming centre a tract also passes to Broca's centre, and 
destruction of this tract will produce word-dumbness. It will also be 
caused by lesion of the centre for word hearing, as the auditory verifica- 
tion of words used will not be possible. Certainly the symptom will 
result from lesion of Broca's centre, to which all tracts concerned with 
speech on its sensory and concept sides pass. It is scarcely necessary to 
say that anomia will be present if a lesion destroys the tracts from the 
retina to the basal and cortical centres for sight. Lateral hemianopsia 
is frequently combined with word-dumbness, because of the juxtaposi- 
tion of the optic radiations to the tracts and centres which are concerned 
with object recognition and the processes associated with it. Destruc- 
tion of the macular tracts and centres will, of course, interfere with the 
recognition and the naming of words, letters, and other objects. 



CHAPTER IV. 

GENERAL EXAMINATION OF THE EXTERIOR OF THE EYE 
AND THE REGION OF THE EYE; PERIMETRY; COLOR- 
BLINDNESS; PERIPHERAL BLINDNESS, AND SUBJEC- 
TIVE VISUAL SENSATIONS; AMBLYOPIA. 

By CASEY A. WOOD, M.D. 

Inspection of the Eye. — Much valuable information concerning 
the condition of the eye, not only of its external but of its internal struc- 
tures, may be obtained by simple inspection. This is especially true of 
an examination made for neurologic purposes ; there are many valua- 
ble signs of involvement of the nervous apparatus exhibited by the 
external eye that may easily be detected if the observer will take a little 
trouble. Such an examination must, of course, be conducted at close 
range and may be aided by instruments. To be effective it should be 
methodical and a certain order ought to be preserved in all cases. Great 
gentleness should be observed and as little handling of the lids and 
globes indulged in as possible, lest eyes already sensitive be rendered so 
irritable that further examination of them is rendered difficult, pain- 
ful or impossible. 

This objective examination of the eye should begin by seating the pa- 
tient in a chair facing a window admitting indirect or diffused sunlight. 
The surgeon should then carefully inspect the superficial structures and 
parts surrounding the globe. The lids should first be examined closed. 
Do their edges approximate readily, entirely, and without apparent effort 
on the part of the patient ? Does the dermal furrow describe its natural 
semicircle from one canthus to the other ? Can the patient shut both eyes 
tightly, and does the wrinkled skin present about the same appearance 
on both sides ? Is he able to open both eyes to their fullest extent and 
in doing so is one eyebrow drawn upward as high as the other ? Is there 
contraction, tonic or clonic, of the orbicular muscle ? On again assum- 
ing the primary position is there drooping of either lid ? The lids in 
normal eyes should be in close apposition to the globe, and the small 
puncta lachrymalia should remain in intimate contact with the eyeball 
in almost all excursions of the latter. The margin of the upper lid 
should rest on the cornea, and this relation should be preserved in the 
downward movement of the ball; in other words, there should be no 
152 



EXTEKIOR EXAMINATION OF THE EYE 153 

exposure of the white sclera between the upper lid-edge and the cornea 
proper. 

Incidentally, one may examine the lids for irregularity of their out- 
line, for oedema, distended vessels, redness, tumors, etc., and for crusts, 
discharge or ulceration along their margins and intermarginal spaces. 







Fig. 1.— Everting the lower lid. (From Wood and Woodruff.) 

The eyelashes also should be inspected, as to whether they are lacking, 
are abnormally numerous, misplaced, incurved, etc. 

The position and sufficiency of the lachrymal drainage should be 
determined. If there be an overflow of tears its meaning should be 
decided; or as sometimes happens, to a paresis of the nerve-supply to 
the orbicularis ; whether it be due to obstruction in some part of the 
lachrymal passages. 

Both lids should be everted and the condition of the conjunctivae, 
ocular and palpebral, determined. To do this thoroughly, and without 
hurting the patient, the tip of the thumb should be placed on the lower 
lid near its margin and the patient told to roll the eye upward. As he 
does this the lid is pressed backward, exposing the whole extent of the 



154 



THE EYE AND JSTEKVOUS SYSTEM 



lining mucous membrane. Complete eversion of the upper lid is more 
difficult. The examiner stands behind the chair on which the patient 
sits, and the latter is directed to look down during the whole perform- 
ance. The forefinger tip of the left hand is now placed upon the upper 
lid just below the eyebrow and the lid drawn by the finger up and away 




Fig. 2.— Everting the upper lid, first act. (From Wood and Woodruff.) 



from the globe so that the lashes point upwards. The cilia and lid edge 
are now grasped by the forefinger and thumb of the right hand, care 
being observed not to touch or press upon the eyeball. The lid is now 
drawn steadily down and away from the globe while the tip of the left 
forefinger is again slipped to the upper margin of the tarsus and pressed 
downward and backward upon the globe. At this moment the lid may 
be readily turned back over the nail of the forefinger, exposing the upper 
sulcus with its covering of conjunctiva. 

The position of the eyeball in the orbit can usually be determined at a 
glance, although when there is swelling of the lids and periorbital struc- 
tures the relations of the globe to the orbital margins, or of one eye to 
the other, cannot always be determined in this way. 



EXTEKIOK EXAMINATION OF THE EYE 



155 



Proptosis, or abnormal protrusion of the eyeball, is noticeable only 
when one eye is more prominent than the other. It is usually by com- 
paring one orbital region with the other that the degree of exophthalmus 
is determined. Where both eyes are unusually and equally prominent 
it is difficult to express such a proptosis in adequate terms. There are 
so many people who have jmysiologically prominent eyes that it is diffi- 
cult to do more than express the degree of protrusion in relation to sur- 
rounding structures. Let the patient be placed in the primary position, 




Fig. 3.— Everting the upper lid, second act. (From Wood and Woodruff.) 

when a straight edge, touching the cheek and supraorbital ridge, is placed 
over each globe parallel to and equidistant from the nasal crest. The 
distance between the front of the cornea and the straight-edge in each 
eye can be accurately stated in millimetres. In some instances, not nec- 
essarily pathologic, both cornea project beyond such a measuring stick, 
so that this simple test cannot be applied. In these cases the author has 
found the ophthalmometer of some value, for the purpose of determining 
whether one eye is the subject of a true exophthalmus or not. The 
patient's head being accurately and rigidly placed in the chin- and head- 
rest, the images of the mires are distinctly reflected from one cornea and 
the distance from the external canthus of the corresponding eye to the 



156 



THE EYE AKD NEKVOUS SYSTEM 



central point at the end of the telescope carefully measured. The 
observation is repeated on the other side. The difference between the 
two measurements expresses the amount of proptosis in the more promi- 
nent eye. 

Various proptometer have been devised, but none of them furnishes 
more useful information than the methods just described. 



<r^^ 




Fig. 4.— Oblique or focal illumination. ( From Posey and Wright.) 



Oblique Illumination.. — The anterior segment of the eyeball is most 
satisfactorily and thoroughly explored by the aid of a convex lens, of 
about six centimetres focus, and a source of light in a darkened room. 
The rays from an argand burner or an electric lamp, placed about a 
metre to the side and a little to the front and above the patient's head, 
are allowed to fall on the eye looking straight in front. The lens is 
held so that the rays of light fall at right angles upon it and are 
focused obliquely on the eyeball. The eye is now moved in various 
directions so that the whole surface of the cornea and globe is illu- 
minated by the pencil of light. In this way not only the external struc- 
tures of the globe but the iris, anterior chamber, pupil, and lens are thor- 
oughly illuminated and explored without producing any of the trouble- 
some reflections that embarrass the observer when the rays fall upon the 
globe from a source of light situated directly in front of the patient. The 
value of this examination is enhanced by placing before the observer's 
eye another magnifying loop of the same focus, with which to inspect 



EXTEEIOE EXAMINATION OF THE EYE 157 

the area lighted with the aid of the other lens. This lens may be held 
in front of the observer's eye with one hand while the other directs the 
illuminating rays upon the corneal surface (Fig. 4). 

Field of Vision. 

There is no examination of the ocular functions of greater value to 
the neurologist than the exploration of the extent and other characters 
of the visual field. Although the test is a subjective one and dependent 
somewhat upon the intelligence and cooperation of the patient it is, 
when undertaken with proper care and a due regard to the requirements 
of each case, difficult to overestimate the assistance it affords in diag- 
nosis. The author entirely agrees with Wilbrand 1 that " the measure- 
ment of the visual field and the registration of the results in the form 
of a map have made it possible to represent graphically the intensity 
and extent of existing visual disturbances and to compare these pictures 
with one another. It can be proved that certain defects in the field of 
vision correspond with certain disease-processes. Moreover, a more 
precise diagnosis has been rendered possible by this method in lesions 
of those parts of the optic tract in which the ophthalmoscope cannot 
afford any assistance. Perimetry has obtained a still greater diagnostic 
importance, since careful study of the results of microscopical examina- 
tion, with exact sketches of the field of vision, has widened our knowl- 
edge about the partial course of the fibres in the optic tracts, and seems 
about to fix the distinguishing symptoms under which neuritis, primary 
atrophy, and purely functional disturbances make their appearance in 
the visual field, as well as with regard to their prognosis and treatment." 

On the other hand, it must be equally remembered that hasty and 
injudicious measurements of the visual field are likely to be valueless, 
misleading and harmful. 

Various forms of the instrument (perimeter) used for determining 
the extent and character of peripheral vision have from time to time 
I)een devised, but, for all practical purposes, they may be divided into 
two classes, first, those intended for patients who can sit upright during 
the examination without much general or ocular fatigue ; second, those 
by which the measurements are taken Avhile the patient is in the prone 
position. The usual form of the perimeter employed in the first class 
of cases is that devised by McHardy, Landolt, Skeel, and others. The 
author prefers the first mentioned. It consists of a semicircular arc or 
band which revolves upon its centre so that it can be placed in the direc- 

1 Norris and Oliver, System of Diseases of the Eye, vol. ii. p. 191. 



158 THE EYE AND NERVOUS SYSTEM 

tion of any meridian. This arm is divided into degrees, beginning with 
at the centre and extending to 90° at either extremity. At the centre 
of the semicircle is a small object {fixation point) for the eye to look 
at. The patient's head is supported by a chin-rest, one eye is covered, 
and the other, under examination, directed to look at the fixation point 
at the centre of the semicircle. A small white or colored object, vary- 
ing from 5 to 20 millimetres square, is then carried from the periphery 
along the inner surface of the arc. The point at which it first comes 




Fig. 5.— The McHardy Perimeter. (From Posey and Wright.) 

into view in each meridian denotes the limit of peripheral vision for that 
meridian. These points (marked on a chart) when joined by lines give 
the boundary of the field of vision. If any defect is found during the 
examination the movable objects will appear blurred or disappear en- 
tirely. This defect is known as a scotoma. 

A 'physiological scotoma is present in every field of vision. It cor- 
responds to the optic nerve entrance and is situated about 10° to the 
outer side of the point of fixation, and is known as the blind spot of 
Mariotte. When a perimeter cannot be obtained the field of vision may 
be roughly measured by the hand in the following manner : the patient 



EXTEEIOE EXAMINATION" OF THE EYE 159 

and the examiner face one another, the former with his back to the light. 
If the left eye is to be tested, the other eye is covered with a towel. The 
examiner closes his right eye and each looks into the other's eye at a 
distance of about a metre. The hand is then carried to the periphery of 
the field at an equal distance between the patient and himself. It is 
then moved slowly inward towards the visual axis and the patient is 
required to say when he sees the finger-tips. This is repeated in the 
various meridians of the field of vision. If the patient sees the fingers 
at the moment they are also seen by the examiner, his field of vision is 
normal. If the fingers are not seen until they are nearer the visual axis 
than when seen by the examiner, there is some defect in the patient's 
field of vision. The perimeter-arc and the test objects are best illu- 
minated by ordinary, diffuse daylight from a window, to which the 
patient presents his back. When that is not obtainable gas or electric 
lamps may be employed, care being observed that the illumination is 
uniform in the various positions of the arm of the perimeter. 

Myles Standish employs, as a rough test for absolute defects in the 
visual field, a small electric lamp and conducts the examination in a 
dark room. One eye of the patient is completely excluded by means of 
a folded towel held over the orbital region by the palm of the patient's 
hand while he fixes the observer's nose with the uncovered eye. An 
electric lamp, of the smallest size, is employed as the test-object instead 
of the finger-tips in the preceding test. 

Sweet has devised a perimeter in which the objects are white and 
colored electric lamps, but the writer has not used it. 

On every perimeter chart used for mapping out a field of vision, a 
note should be made roughly stating the kind and amount of illumina- 
tion employed, such as " bright day," " dull daylight," " electric light," 
etc., as the size of the field is, in some instances, much affected thereby. 

Another rough means of defining the limits of the field of vision is 
the ordinary school blackboard. Although theoretically insufficient it 
is a fairly good substitute for the perimeter. In such cases black rods 
with round or square tips, or pieces of chalk, white and colored, are 
employed as test objects. While the patient fixes a white chalk mark 
on the blackboard, these objects, moved about as on the arc of the perim- 
eter, map out the visual field. 

The portable 'perimeter of Wilbrand, 2 or one like it, is valuable in 
the examination of bedridden patients. It is used in the wards of a 
hospital or on occasions when the stationary instrument is not accessible. 

2 Norris and Oliver, System of Diseases of the Eye, vol. ii. p. 210. 



160 



THE EYE AKD KERVOUS SYSTEM 



The inventor terms it a bed- perimeter. "A wooden board (a), the breadth 
of which is equal to the length of the perimeter, and the length of which 
equals the width of the bed, is made so that it can be raised or lowered 
like a reading desk. A second board (&), which can be placed at any 
degree of inclination upon two perforated irons (c), is supported by 
putting a little iron pin (d) into a series of holes. Upon the board, b, 
is placed the perimeter. The board, b, is connected with the board, a, 
on the side turned towards the patient, by means of two hinges, which 
are placed near the corners. Both boards between the hinges are hol- 
lowed out so as to fit the regions of the chest and abdomen. The perim- 
eter itself is supported below by a clamp (/) , which is fixed vertically 




Fig. 6.— Wilbrand's bed-perimeter. (From Norris and Oliver. ) 



to (b). The board (a) is supported by four wooden screws (g), which 
are fastened by pairs to a rather narrow board (h) resting horizontally on 
the lateral bed-boards. On the board Qi), near the outer end is placed a 
clamp (&), so as to prevent the whole apparatus from moving sideways. 
A head-rest is so arranged that the chin of the patient can be brought into 
a comfortable position on the chin-rest of the instrument." 

The best results in perimetry can be obtained only by the exercise of 
much patience and the expenditure of considerable time. Many neu- 
rologic patients after a short examination exhibit the so-called " retinal 
fatigue" and many others have such dull peripheral vision that the limits 
of their fields, especially for colors, are not easily defined. Again, it is 
not an easy task for either the examiner or the examined to locate and 
measure scotomata, particularly if they are small or incomplete. Ere- 



EXTEKIOK EXAMINATION OF THE EYE 161 

quent pauses should be made during the examination, so that the patient 
may rest his eyes, and if fatigue-signs are marked the completion of the 
examination should be deferred until another sitting. Unfortunately, it 
is in just such instances that control experiments should be made, that 
is to say, it is in cases exhibiting a " fatigue" neurosis that one must be 
most careful to repeat his observations and where it is desirable that 
there should be no " nerve-tire" produced by the efforts of the physician 
to conclude an examination. The patient should be encouraged to close 
his eyes, sit back in his chair, and rest himself as often as need be. 

These difficulties are further increased when, for any reason, the 
patient is unable to see the fixation point on the perimeter. It is then 
advisable to have him place the tip of his forefinger over the point of 
fixation and, by virtue of his sense of locality, keep his eye in the 
primary position. The examiner must watch him closely to see that 
his gaze does not wander. 

Conditions that Determine the Limits and Other Characters of 
the Visual Field. — It is particularly desirable to remember, in examin- 
ing neurologic cases, that a number of factors enter into the results 
obtained by a perimeter and that many conditions may modify the shape 
and area of the field. Our standard should be the extent and boundaries 
of the visual field in the normal, emmetropic eye. Although, as before 
stated, the examination is essentially a subjective one we cannot always 
obtain the same results in every measurement even in the same patient 
or from observations of the same eye, but the error is generally so 
slight that it can be ignored, or at least borne in mind when considering 
the total value of the evidence presented by this and other means of 
diagnosis. 

The shape of the normal visual field is an irregular oval, due to the fact 
that the retina extends farther forward on the nasal than on the temporal 
side, thus pushing the limits on the perimeter-map correspondingly out- 
ward. 

The boundaries of the field of vision are also affected by the promi- 
nence of the eyeball in its socket, by the tip and bridge of the nose, the 
cheek and even by the size of the lips and moustache. They are often 
measurably widened by opening the eyes to the fullest extent, and there 
is no doubt but that, other things being equal, a larger field is found 
with a dilated pupil than with a narrow one. Owing to the long antero- 
posterior axis in myopia and the shorter optic axis in hypermetropic the 
field is smaller in the former and larger in the latter case. Bearing these 
facts in mind one should always have the patient under examination 
wear his correcting lenses and not hesitate to secure a full measurement 
11 



162 



THE EYE AND NERVOUS SYSTEM 



of the field by asking him to open his eyes wide, or separate his lids 
with the fingers. The other difficulties may to some extent be overcome 
by throwing the head first backward and then inward during the exam- 
ination of the vertical and horizontal meridians respectively. 

The extent of the field for color varies with the character of the pig- 
ment used, the size of the object, the degree of illumination, the nature 
of the background against which it is displayed and with individual 
peculiarities. For example, women, with a more acute perception of 



Bight Eye 



70 






X 



^ 



V 40 



io_6.o Ao _lo_ $ 7|r- 

x \ x 



x \ 






A- 






vOkF \ 



%/ 



■/. 1, 






/^ 



/> 






— +■ 



^ 



Fig. 7. —Normal field of vision, right eye. 



white : 



blue ; 



red 



green. 



(From Posey and Wright). 



color, have larger color-fields than men. For these reasons the charts 
found in most text-books exhibit considerable variations as to the extent 
of the color-fields. Yellow always has the largest area in the chart of the 
normal eye ; then comes blue, then red, then green. 

Although, in the normal eye, the size of the field for white (taken 
with a five-millimeter object) varies little with the illumination, quite 
the opposite is the case when color tests are used. . This fact should 
especially be remembered in testing for nervous disorders. 

Variations in the size of the field for white in pathologic states with 
different degrees of illumination are not infrequent. We see this espe- 
cially in the different forms of hemeralopia as encountered in retinitis 
pigmentosa, disseminated choroiditis, high degrees of myopia, secondary 
glaucoma, luetic chorio-retinitis and the like (See Chapter VIII). 



EXTEEIOK EXAMINATION OF THE EYE 163 

Much has been written upon the need of standardized color-objects 
for the perimeter and, doubtless, greater scientific accuracy would be 
attained if these always exhibited the same color intensity or satura- 
tion. Colored test-objects should be as small as is compatible with dis- 
tinct vision; in cases of amblyopia they may be as large as 20 milli- 
metres square. It is well to remember that scotomata, especially of 
limited area, are best mapped out by the use of much smaller objects, 
whose sides measure only two or three millimetres. 

The author has long been in the habit of using a single strand of 
white, red, green and blue wool for detecting the presence of central 

Left Eye 



X 



X 



X s 



V 



•-/^, 



. / ■ 



>r" 



X 



■< 



v ^ 



A 



-4AJ 



<jyx 






035 



V 









•fTlS 



:>sx 



>v 



<\ \ 



"XCx 



* \ \x-\- 



:V¥ 



~3R 



v 40 50\ „„ 



150\ 



;v-- 









X 




\sAK?¥j^tS/j it 



Fig. 8.— Normal field of vision, left eye. 



white : blue ; 



(From Posey and Wright. \ 



red : 



color scotomata. Double the thread between the thumb and finger of the 
right hand, holding it so that only a quarter of an inch of the loop pro- 
jects and is seen. Patient and surgeon should be seated opposite 
one another. Cover the patient's left eye with a closely-fitting shade, 
and ask him to ^x steadily the observer's right pupil at a distance of 
two-thirds of a metre. Interpose the small loop of wool in the common 
line of vision at a point a few centimetres in front of the examiner's 
eye. In relative central scotomata the patient will not recognize the 
color of the small loop (or it will appear less distinct) until it is removed 
a few millimetres to the right, left, up or down from the line of sight, 
and the distance it is necessary to carry the test loop in the various 



164 



THE EYE AKD NEKVOUS SYSTEM 



directions before the color is recognized will give an idea of the size and 
shape of the scotoma. This rough test should then be applied to the left 
eve, after which the defect so detected can be properly charted by means 
of the perimeter. 

The size of the field to some extent depends upon the manner in which 
it is measured — another argument in favor of always making perimetric 
examinations under conditions and in a fashion as closely resembling one 
another as possible. A uniform plan will give definite information and 
satisfactory results, while haphazard and varied measurements will only 
confuse and discourage the observer. Eor example, it has long been 



Left Eye 




90 75 

Fig. 9.— Central scotoma as seen in toxic amblyopia. (From Posey and Wright.) 



known that, in pathologic cases especially, there is a zone of doubtful 
form- and color-perception about the boundary line of most fields. 
Within this zonular region both the observer and the intelligent patient 
remark of the test-object that ." now he sees it and then he doesn't see 
it." This must be borne in mind when repeated examinations are made ; 
also that with ocular conditions unchanged the patient will (within 
certain limits) report a gradually widening area of color- and white-per- 
ception the more frequently he is examined. To a slight extent practice 
increases the ability of the patient's perceptive elements. 

The field is larger when the test-object is moved from the centre to the 
periphery than when it is carried in the opposite direction ; or when the 



EXTEKIOK EXAMINATION OF THE EYE 



165 



object is " wiggled," or kept in motion, about the zone of doubtful per- 
ception. Of course, it is wider when a large, shiny object is employed 
than when a dull, small one is used. 

Central scotomata result from disease of the macular fibres. These 
are termed absolute central scotomata when all light perception is want- 
ing. Defects for one or more colors only are called relative central 
scotomata. When white is seen as " grayish," or colors are appreciated 
only as tints of the test-object, the defects are known as inaccurate cen- 
tral scotomata. Finally, Forster adds the terms positive and negative 
central scotoma, defining the former to be that condition in which the 
patient reports a dark spot covering the fixation-object and the latter 
when the point of fixation appears blurred or indistinct. The precise 
conditions of the central field should always be indicated on the perim- 
eter chart or noted on its margin. 




Fig. 10— Ring scotoma. (From Norris and Oliver.) 

The foregoing, distinctions are now and then valuable in diagnosis, 
because negative central scotomata are generally the result of disease of 
the papillo-macular bundle of optic nerve-fibres, while positive defects 
are generally due to peripheral alterations in the macular region, often 
chorio-retinitic changes. Among the numerous forms of central scotoma 
an important one for the neurologist to remember is that due to " retinal 
fatigue," not infrequently seen in functional nervous diseases (See 
Page 108). 

The position of central scotomata with reference to the point of 
fixation is indicated by the terms paracentral, when the defect lies mostly 
without the centre, and pericentral, when the fixation-point forms the 
centre of the defect. 



166 



THE EYE AND KEKVOUS SYSTEM 



Enlargement of the blind or Mariotte's spot must not be overlooked in 
searching for defects in the visual field. This may be due to congenital 
conditions, such as the presence of opaque nerve-fibres, but it is also one 
of the early signs of certain forms of optic atrophy, of chorio-retinitis 
of the fibres surrounding the nerve-head and it is seen in most cases of 
" choked disk." 

Ring Scotoma. — The area lying between the fifteenth and forty-fifth 
parallels of latitude is called the intermediate zone. It is occasionally 
subject to fairly well defined defects, which, enclosing an otherwise 
normal central field of zone, constitute a " ring" scotoma (Fig. 10). 




§ S 

Fig. 11.— Defect confined to the peripheral zone of the visual field. ( From Norris and Oliver.) 



This annular defect is sometimes found, of varying width and shape, 
in syphilitic lesions of the chorioid and retina, in multiple sclerosis of 
the optic nerve and a few other conditions. 

The peripheral zone of the visual field may be defined as that lying 
between the forty-fifth parallel and the extreme limits of the field. Wil- 
brand says that it generally begins in a normal eye where the perception 
of a five-millimeters blue object ceases. Isolated defects in this area do 
not disturb the vision of the patient to any extent, and we are mostly 
concerned with it in the diagnosis and prognosis of optic and other 
nervous affections. When the whole or most of the peripheral zone is 
insensitive to light patients realize the value of indirect peripheral 
vision. They act and feel as if they were seeing through a tube, and 
when the contraction extends still further towards the centre " telescopic 
vision" is the result, obliging the one so afflicted to look about him con- 
tinually to obtain satisfactory sight (Eig. 11). 



EXTEEIOR EXAMINATION OF THE EYE 



16' 



Hemiopia, or (better) Hemianopsia, is characterized by loss of one 
half or portions of half the field in each eye. It may result from disease 
in any part of one optic conducting path from the calcarine fissure to 
and including the chiasma (Figs. 13, 11). It is almost invariably 
homonymous, — i.e., affects the optic radiations supplied to the two eyes 
from one side of the brain without involvement of either fixation-point. 
Binasal, bitemporal, or heteronymous hemianopsia is quite rare. When 

Left Eye 

90 




Fig. 12.— Scotomata as seen in disseminated chorioiditis. (From Posey and Wright.) 



one considers for a moment the anatomic arrangement of the crossed 
and uncrossed fibres that constitute each optic nerve the explanation of 
homonymous hemianopsia is easy (See Chapter I). 

Of 154 cases of homonymous hemianopia collected by Wilbrand 71 
were on the right side and 80 on the left. The line dividing the blind 
from the seeing half of the retina does not, as a rule, pass directly 
through the fixation-point. Fortunately, the functionating half usually 
includes the fixation-point, — i.e., the lesion does not affect the fovea, 
because there are sufficient unaffected fibres from the normal visual 
centre supplied to each papillo-macular bundle to permit of good central 
vision. Inasmuch as the arrangement and distribution of the crossed 
and uncrossed bundles varies greatly in individuals the position of the 
frontier-line also varies accordingly. As a rule, this boundary runs in 
an almost perfectly vertical direction until it approaches within 5° to 
10° of the fixation-point. It then diverges, curves around the central 



168 



THE EYE AND KEKVOITS SYSTEM 



point and at the same distance below once more resumes its vertical 
direction. The functionating area about the point of fixation may have 
a considerable radius — from 3° to 10 °, or even more. 

The variations in the line dividing the seeing from the non-seeing 
retina correspond to variations in the size of the hemiopic fields, only, as 
a rule, the same part of the field is affected in each eye even if they vary 
much in extent. Later, part of the field may be recovered on one side 
while undergoing no change in the opposite eye, rendering the contrast 
between the two perimeter charts still more marked than before. 



RightEye 



x y^ A" 



M-W-WcO 40 3b 2<T- 

i T T - r~T— t-- 

150' 





90 75 

Fig. 13.— Field in left-sided hemianopsia. (From Posey and Wright.) 

Incomplete hemianopsia is due, in the majority of instances, to 
destructive lesions of the occipital lobe. Defects confined to either the 
upper or the lower quadrants of the field indicate destruction of the 
lower or upper elements of the cuneus (Figs. 15, 16). 

The periphery of the unaffected half of the field in hemiopia may or 
may not be contracted and the half-field is usually smaller on the side 
opposite the lesion (See Chapter IX, p. 391). 

The field of vision in binocular fixation is, of course, larger than that 
of each eye taken separately. Although it is the field generally consid- 
ered by the patient and ought, consequently, to be of particular interest 
to the neurologist, its practical value, apart from the subject of squint, is 
not very great. 

Hemichromatopsia. — In true hemianopsia perception of color is lost 



EXTEEIOK EXAMINATION OF THE EYE 



169 



with the field for white. In rare cases, however, there is a true liemia- 
chromatopsia, in which the white field is unaffected (or white objects are 
seen as gray), the dividing line passing through the fixation-point. In 
explanation of this curious condition Wilbrand claims that there is a 
centre for colors distinct from that for form or from that for white. 
Gowers suggests that instances of hemichromatopsia would be more 
frequently noted if the color fields were tested at the bedside in all cases 
of cerebral disease (See Page 392). 

An admirable summary of the diagnostic values of defects in the 
visual field is given by Gowers. 3 Loss of the pupillary reaction with 

Left Eye 




Fig. 14.— Field in left-sided hemianopsia. (From Posey and Wright.) 



defective sight in one eye without affection of the opposite organ indi- 
cates disease of one optic nerve. A central scotoma usually means dis- 
ease of the macular bundle of optic fibres, either in the body of the 
nerve or at the fovea itself. Peripheral limitation of the field may be 
due to (a) damage to the peripheral layer of the nerve or (b) a lesion 
of the whole nerve, which shows itself only in the periphery, where 
vision is normally duller than it is about the macular region. A sector- 
like defect, confined to the field of one eye indicates destruction of a 
portion of one nerve. An amblyopia, with concentric limitation of the 
field, marked in one eye but slight in the other, is due either to atrophy 

8 Diseases of the Nervous System, vol. ii. p. 162. 



170 



THE EYE AND NERVOUS SYSTEM 



or to disease in one hemisphere. In the first instance the pupillary 
reaction is sluggish and the ophthalmoscope generally reveals the 
atrophic changes ; in the second the nerve appears normal, the pupil acts 
promptly to light, the onset of the visual disturbance is sudden and there 
are usually other symptoms of cerebral involvement. In functional, or 
hysterical, amblyopia the symptoms are the same as in the second in- 
stance, but there are other hysterical stigmata present. In those rare 
cases of monocular hysteria the perfect reaction of the pupil serves to 
distinguish the functional affection from organic disease of the nerve. 

Right Eye 




105 i 7S 

Fig. 15. — Homonymous quadrant hemianopsia. (Posey and Wright.) 



Temporal hemianopsia means disease of the chiasma. Complete blind- 
ness of one eye with loss of the temporal field in the other generally 
points to disease of the chiasma extending to the outer fibres of the nerve 
or optic tract on the side on which there is complete loss of vision. 

Wilbrand's or Wernicke's Light-test — the so-called " hemiopic 
pupil-reaction" — is useful in determining whether the lesion in a case 
of lateral hemianopsia lies in the tractus opticus or in the cerebral cen- 
tres behind it (Fig. 17). The test depends upon the fact that when light 
is thrown upon the blind half of the retina the pupil contracts just as 
promptly as it does when it is projected upon the seeing half of the dis- 
ease confined to the cortex or basal neurons. When the tract is involved 
— i.e., when the conducting path to the corpora quadrigemina is diseased 
— it does not contract. 



EXTEEIOE EXAMINATION" OF THE EYE 



171 



The patient should sit (as for an ophthalmoscopic examination) in a 
half -lighted room with one eye carefully covered and fixing with the eye 
to be tested an object directly in front, at least ten feet away. The rays 
from a small electric tube-lamp should be first focused on the forehead 
and then allowed to strike only the blind half of the retina. The effect 
is noted and compared with the result obtained by illuminating in the 
same way the other retinal half (See Page 393). 

The ZEsthesiometer is of considerable value in the examination of the 
eyes of neurologic patients. Variations in the tactile sensibility of the 
palpebral skin are best tested and mapped out by either Carroll's or 
Sieveking's instrument, but conjunctival and corneal areas of anaesthesia 
•or hyperesthesia cannot, for obvious reasons, be so measured. The 




Fig. 16.— Homonymous quadrant hemianopsia. (Posey and Wright.) 

author has found that a small camel's-hair brush answers every useful 
purpose. Employed with a little care, particularly where it is possible 
to compare a normal with an abnormal eye, valuable information can 
loe obtained, not only in functional affections — mostly hysterical — but 
in those organic lesions — paretic and other — that reduce or increase the 
conjunctival and corneal sensibility. In examining the patient with this 
end in view, it is well to have him rotate the eye upward as much as 
possible, while very gently touching the lower conjunctival surfaces and 
lower half of the cornea with the tip of the brush. Then, the upper lid 
should be everted and the exposed conjunctiva? and upper hemisphere of 



172 



THE EYE AND NERVOUS SYSTEM 



the cornea are similarly tested. In this way the nervous patient does 
not see the approach of the brush-tip and is obliged to regulate his 
answers by touch-sensations only. 

Color-Blindtstess. 

Color-blindness is either congenital or is due to some organic change 
in the optic nerve-fibres or the visual centres from which they spring. 
The latter class is best illustrated in toxic (tobacco or alcohol) ambly- 
opia, optic atrophy, diabetes, hysteria, etc. Congenital anomalies of the 
color-sense, due to organic defects in some element of the optic radia- 
tions, are more common in men (four per cent.) than in women (one 
per cent.). (See Page 92). It is well to remember that while this con- 
genital defect cannot be remedied and that the person born color-blind is 




Fig. 17.— Illustrating the test for hemianopsic pupillary inaction ; the lines represent a horizontal 
plane through the left eye and its visual field : F 0, fundus oculi ; M, macula lutea ; X, nasal half of the 
field which is anaesthetic in temporal hemianopsia ; T, temporal half of the retina ; T F, temporal 
field ; P, pupillary aperture, 180° to 0°, the equatorial arc or semicircle ; 90°, vertical point and line 
passing through centre of eye to M ; 70° and 40°, rays of light striking the insensitive nasal half of the 
retina, producing the pupillary reflex. (Seguin. ) 

always color-blind, the intelligent individual establishes a standard of 
color for himself and so frequently discounts some of the drawbacks that 
reside in a dyschromatopsia. Occupations like that of railroad fireman 
or engineer, requiring acute perception of colors, should not, of course, 
be undertaken by color-blind individuals. 

Color-blindness is, for clinical purposes, readily detected by the use 
of various colored skeins of wool after the plan devised by Holmgren. 
Thomson's " color-stick" (Plate I) constitutes a handy arrangement 
of the skeins, which are numbered to correspond with a private key 
for the use of the color-blind examiner. The patient is seated before a 
table with, preferably, a sufficient natural illumination from a side 



:. >: . - 




2 nni 

- 




EXTEKIOK EXAMINATION OF THE EYE 173 

window, on which are placed skeins of colored wools in different shades 
of red, green, gray, brown, etc. In front of him is a large skein or knot 
of pure green wool. This is the " test" skein, and he is required to select 
from the others in front of him the skeins of the same color, but not 
necessarily of the same shade. If there is any defect in his color vision 
the patient will select a number of the confusion colors (chiefly grays), 
with perhaps some green tints. If he accurately chooses all greens the 
investigation need proceed no further; he has normal color-sense — is 
not color-blind. If, however, he fails, he is given a rose-colored skein 
and again told to choose wools of that color. He is now likely to match 
the rose with browns, purples, dark grays or even greens. Finally, to 
find out what variety of color-blindness he has, he is given a deep red 
skein, to match which he will probably choose the dark browns and 
greens. The great majority of color-blind people are defective in red 
and green and, consequently, are said to have red-green-blindness, 
although all varieties, from simple green to total color-blindness, exist. 

With Holmgren's test it is almost impossible for one who is color- 
blind to escape detection. Thomson's modification of this test is prob- 
ably the most convenient. The person under examination should not 
be allowed to name the colors ; only to select those colors resembling the 
test-skein in his hand. 

Peripheral Blindness. — Generally speaking, blindness is regarded 
as that loss of sight that incapacitates the individual from following any 
occupation requiring the use of the eyes. The proportion of blindness 
due to peripheral causes — i.e., those affecting the external organs of 
vision — is large. The tables of Magnus show that at least 67 per cent, of 
2528 cases occurring in Germany were due to idiopathic eye affections. 
On the other hand as high as two per cent, resulted from purely cerebral 
diseases ; injury, congenital defects and general affections being account- 
able for the remainder. Gonorrheal infections of the conjunctiva (oph- 
thalmia neonatorum, gonorrheal ophthalmia of the adult), trachoma and 
diseases of the cornea induce blindness chiefly by producing scars in the 
clear corneal tissue. These infections were alone responsible for blind- 
ness in 27 per cent, of Magnus's cases. Spinal optic atrophy as well as 
irido-chorioiditis from meningitis (particularly the epidemic cerebro- 
spinal variety) are common causes of lost vision. 

When sight is lost from destruction of the cornea the necrosed tissue 
is replaced by cicatrices (leucomata especially) that are readily detected 
by the naked eye. More deeply seated opacities of the media, such as 
cataract, are better seen by the aid of the lens and the oblique illumina- 
tion, already described on page 156. The use of the op/itlialnwscopc is 



174 THE EYE AND NERVOUS SYSTEM 

required for the identification of many lesions of the posterior structures 
of the globe that with equal certainty produce blindness. Diseases of the 
chorioid, retina, and optic nerve, changes in the vitreous body, intra- 
ocular growths, glaucoma, — all these generally require an exploration of 
the posterior chamber and fundus oculi through the pupil for a positive 
diagnosis. The employment of the ophthalmoscope in the determina- 
tion of sources of blindness is all the more imperative since the uveal 
tract, optic nerve, and retina constitute the anatomical seats of more 
than half the blindness one meets with. 

Subjective Visual Sensations. 

Peripheral Subjective Visual Sensations. — Muscae Volitantes; Phos- 
phenes. — The appearance of specks, spots, or other minute objects float- 
ing in front of the eye may or may not depend upon organic disease of 
that organ. In most instances musca? are due to a hypersensitive per- 
cipient apparatus which takes notice of the normal opacities in the vitre- 
ous, but it is not uncommon to discover exudates and other floating 
deposits within the vitreous whose presence easily accounts for these 
appearances. 

Phosphenes are colored rings or flashes of light which may at any 
time be induced by pressing on the inner aspect of the closed eye. These 
bright color sensations may also be caused by cerebral diseases, by optic 
nerve affections, by glaucoma, and by other conditions. 

Hallucinations of Vision. 4 — Although in a restricted sense most 
phosphenes and muscae volitantes are examples of visual hallucination 
they are not usually regarded as such. The sensations of this order due 
to -cerebral diseases are on the other hand mostly genuine delusions, just 
as we know them to be in acute alcoholic mania. Hansell gives three 
varieties of visual hallucinations ; first, those due to ocular disease, 
whether organic or peripheral; second, those that arise from localized 
disturbance of the cerebral circulation, including functional nervous 
affections, and, third, those resulting from organic disease of the cortical 
centres. Purely ocular hallucinations, as Hansell points out, are de- 
pendent upon a number of causes. These he enumerates as follows : ( 1 ) 
errors of refraction, (2) oculo-muscular defects, (3) opacities of the 
dioptric media, and (4) change in the choroid, retina and optic nerve. 
Thus myopes have an inaccurate impression of size, distance, color, and 
detail, and err in their judgments of matters concerning which their only 



4 For more extended information on this subject the reader is referred to the 
recent papers of Drs. Wm. Campbell Posey and Howard F. Hansell. 



SUBJECTIVE VISUAL SEXSATIONS 175 

or chief information comes through their organs of vision. So much is 
left to the imagination in the make-up of a landscape, a picture, a 
human face, that they suffer a rude awakening when made to realize by 
glasses the real appearance of things. The hypermetrope sees the detail, 
but misses the harmonious whole. The astigmatic individual sees the 
square an oblong, the circle an oval, and fails to perceive with equal 
clearness simultaneously all parts of an object (Compare Page 388.). 

The patient with a paralyzed ocular muscle receives two impressions 
of every object, neither of them correct, because the mental conception 
must be formed without the association of the binocular co-ordination 
and physiologic double retina image. The mental impression of opaci- 
ties of the cornea is that of cloudiness ; of opacities of the lens, that of 
a fog; of opacities of the vitreous, that of smoke. Minute scattered 
vitreous opacities are extremely deceptive, and lead the patient to believe 
he sees flies, spiders, webs, that elude his grasp as he reaches to brush 
them aside. 

Irritation of the rods and cones of the retina and the axis cylinders 
in the nerve and retina by the pressure of blood or inflammatory material 
accentuates the normal activity of the tissues and the field of vision is 
occupied by brilliant meteoric displays of flashes and circles of light, 
bright or colored rings or waves of light rapidly passing from one side 
to the other, or emanating from the centre and spreading toward the 
periphery, startling and real while they last, and their disappearance is 
followed by a feeling of intense relief. 

The author has notes of a number of cases in which patients had, 
without any evidence of nervous disease and exhibiting only faint opaci- 
ties of the vitreous or slight choroidal lesions, distinct visions of rats or 
mice running across the room. In every instance these phenomena were 
visible towards one or other temporal side of the patient and at a dis- 
tance of from two to three metres. As they all wore glasses the hallu- 
cination was attributed to images projected into the over-sensitive eye 
from the inner surface of one lens. 

The scintillating scotoma, or " fortification spectrum," of migraine 
is sometimes seen without the occurrence of headache, vertigo or nausea. 
Occasionally it appears as a visual aura of epilepsy. In the first instance 
it may mislead the surgeon into the fear of an approaching optic atrophy, 
but an examination of the visual field and the use of the ophthalmoscope 
will clear the diagnosis. 

Hemeralopia (sometimes called nyctalopia), or day blindness, consti- 
tutes a peculiar hyperesthesia of the retina, in which vision is decidedly 
worse when the patient is exposed to bright daylight, but improves with 



176 THE EYE AND NERVOUS SYSTEM 

a dim illumination. It is sometimes associated with true photophobia, 
phosphenes and with various other visual hallucinations. It is seen, now 
and then, after prolonged exposure to intense reflections from large 
areas of water, snow, ice, etc., and is not uncommon in albinism and in 
those suffering from coloboma of the iris and choroid. 

Nyctalopia, on the other hand, occurs in a sensitive retina. The 
person so affected sees fairly well in ordinary daylight, but the moment 
the intensity of the illumination is reduced vision is much worse. This 
condition is occasionally functional, but is often due to organic disease 
of the choroid and retina, as seen in retinitis pigmentosa, syphilitic 
chorio-retinitis and in some degenerations of the retina not due to lues. 

Erythropsia, or red vision, is one of the most frequent forms of chro- 
matopsia. Colored vision is generally due to the absorption of pigments 
that either tinge the dioptric media or produce some unknown effect 
upon the percipient elements of the retina. Probably this explains the 
yellow vision (xanthopsia) of jaundice, as well as that following poi- 
soning from santonin, amyl nitrite, cannabis indica, picric acid, etc. The 
complaint of aphakic patients that objects seen through the correcting 
lenses are blue, or deeply edged with that color, is not explained by 
the possible prismatic effect of the glasses. Probably the eye, accus- 
tomed to perceive objects through the yellowish medium of the lens, per- 
ceives them as blue (the complementary color of yellow) when the dis- 
eased crystalline is removed. Red vision follows exposure to extremely 
bright white or violet light, for instance, to the rays of the sun or to the 
light from the electric furnace. 

Amblyopia. 

Amblyopia is a term indicating poor vision without apparent cause, 
particularly when the condition is not explained by an ophthalmoscopic 
examination. 

Toxic Amblyopia. — The commonest forms of defective vision pro- 
duced by poisoning are those from the habitual abuse of grain (ethyl) 
alcohol, tobacco in any of its forms, or from the use of both together. 
The disease is usually found in men over forty years of age who suffer 
from intestinal catarrh and who have an idiosyncrasy against stimulants. 
The patient complains of foggy or misty vision and generally applies to 
the ophthalmic, surgeon for glasses to assist his failing sight. Central 
vision often falls to T ? o of normal, and the reading of ordinary print is 
impossible with any lens. Such a patient has a faulty digestion, an 
" old pipe" odor to his breath, anorexia and morning nausea, insomnia 
and dull headache. The ocular condition is mainly due to a central 



AMBLYOPIA 177 

scotoma for red and green, the perimetric examination showing an oval 
defect about the fixation-point. The prognosis in this form of toxic 
amblyopia is generally very favorable, particularly if the patient can be 
persuaded to abandon his toxic habits. 

Rarer forms of amblyopia from poisons are those produced by drink- 
ing methyl or wood alcohol (" Columbian Spirits/' " Cologne Spirits," 
adulterated " Jamaica Ginger," lemon extract, bay rum, etc.), by the 
ingestion of quinine and by the absorption of lead salts. In all these 
cases complete blindness from optic atrophy may occur. 

Reflex Amblyopia is a form of blindness due to irritation (generally 
peripheral) of the nerves in more or less close relation to the optic cen- 
tres. Intestinal worms, especially in children, diseases of the teeth in 
adults and, in rare instances, pathologic changes in the reproductive 
organs have been held responsible for visual defects that have disap- 
peared with the removal of the cause. 

Amblyopia ex anopsia is a term of rather ill-defined meaning. In 
many cases of strabismus due to congenital conditions, or associated 
with them, the squinting eye, although amblyopic, presents no organic 
defect. However, after exercise of the weaker eye (following a removal 
of the barrier to binocular sight) not only central but peripheral vision 
of the eye may very greatly improve. The same statement is occasionally 
true of long-standing early cataract. It is not improper, then, to believe 
that the defective sight of such eyes was due to non-use of the organ — 
just as temporarily paretic muscles may undergo a functional atrophy 
and yet regain their original size on the return of normal innervation. 
It is not yet decided how one may unerringly differentiate between eyes 
capable of visual improvement and those that continue functionally in- 
competent during life in spite of all treatment. 

Amblyopia from Toxins. Uraemia. — Loss of vision from drug poisoning- 
has its counterpart in certain toxic conditions of the blood, particularly 
uraemia. The blindness, as in quinine and methyl alcohol amaurosis, 
comes on suddenly and the pupils are often dilated, although they gen- 
erally react to light. There are no ophthalmoscopic changes due to the 
toxaemia proper, and sight returns when the state of the blood improves. 
Convulsions and other evidence of uraemia usually accompany the visual 
defect. 



12 



CHAPTER V. 

THE EXTEA-OCULAE MUSCLES 

By ALEXANDER DUANE, M.D. 

Section I. 
PHYSIOLOGY OF THE EYE-MUSCLES AND EYE-MOVEMENTS. 
TESTS. NOMENCLATURE AND CLASSIFICATION OF THE 
OCULAR DEVIATIONS. 

Fixation and its Derangements. — Monocular Fixation. — When we 
direct one of our eyes towards an object so as to see it as distinctly as 
possible we are said to fixate or fix that object. 

The fovea centralis is the most sensitive portion of the retina. 
Hence, in performing fixation we normally direct the eye so that the 
image of the object looked at falls upon the fovea. This is called 
central fixation. It may happen, however, that owing to disease of 
the retina or to other reasons vision with the fovea is less acute than 
with some outlying portion of the retina, and in that case the eye, in 
monocular fixation at least, will be directed so that the image shall 
fall upon this outlying portion {eccentric fixation^). 

Monocular Projection. — When we are looking with one eye at an 
object and that object forms its image on the retina, we not only see the 
object, but get an idea that it occupies a certain definite position in space. 
This process by which we infer the position of an object in space from 
the visual impression that the object produces we call projection. 

An object towards which the eye is pointing forms its image on the 
fovea. Conversely, an object which thus forms its image on the fovea 
we judge to be situated in that part of space towards which we think 
the eye is pointing. If our judgments are correct as to the direction 
of our eye, we project — i.e., locate — objects correctly; but if, as in 
paralysis, our notions of the positions of the eye are erroneous, our 
projection will be faulty and we will see objects in the wrong place. 

Not only do we get an idea of the location of an object, A (Fig. 1), 
towards which the eye is pointing, but also a notion, more or less pre- 
cise, of the location of all objects surrounding A. Thus, an object, B, 
that forms its image below the fovea, we regard as situated in space 
correspondingly far above A, for, objects that are actually above the 
178 



THE EXTRA-OCULAR MUSCLES 



179 



point we are looking at do regularly, in our experience, form their image 
below the fovea. So also the object, C (Eig. 2), which forms its image 
to the left of the fovea, we judge to be situated correspondingly far to 
to the right of A. . 

We thus arrive at the law of projection, which may be stated thus : 
An object which forms its image on any point in the retina is projected 
to — i.e., mentally located in — the point in space directly opposite. 

Fig. 1. 





Binocular Fixation. — In the vast majority of cases, if one eye fixates 
an object with its fovea the other will do so too (binocular fixation). 
In fact, the whole extremely complicated mechanism of muscles and 
nerves with which the two eyes are supplied is designed primarily to 
accomplish this single end — i.e., to so move the eyes that they shall 
both be directed accurately to the object we wish to see and each eye 

Fig. 2. 




shall receive the image of that object precisely upon the fovea. The 
movements of the two eyes are hence almost invariably so co-ordinated 
as to secure binocular fixation under all conditions. 

Derangements of Binocular Fixation; Deviations of the Eye. — Normally, 
as we have seen, a person looking with both eyes open fixates the same 
object with both, — i.e., both eyes are " straight." 



180 THE EYE AND NERVOUS SYSTEM 

Not only is this so, but in the majority of cases both eyes will still 
fixate the same object, — i.e., both will be " straight" even when one 
eye is covered. This condition is called orthophoria, and must be 
regarded as the ideal or natural condition. 

In other instances both eyes will look straight at the same object 
when both are uncovered, but either eye as soon as it is covered will 
deviate — turning out, in, up, or down. This condition is called hetero- 
phoria, or, less properly, insufficiency. 

In a third class of cases, comparatively few in number, only one 
eye is straight at a time, the other deviating even when both eyes are 
open. This condition is called squint, strabismus, or heterotopia. 
Squint is further called — 
I. (a) Alternating when sometimes one eye deviates, sometimes the 
other. 
(b) Monocular (right or left) when the deviation always affects 
the same eye. 
Again, the deviation, whether squint or heterophoria, is called — 
II. (a) Intermittent when under the same conditions it is sometimes 
present, sometimes not. 
(b) Constant when it is j)resent all the time. 

III. (a) Periodic when it is present when the eyes are directed at a 

near object and absent in distant vision, or vice versa, 
(b) Continuous when it is present both in distant and near vision. 

IV. (a) Comitant or concomitant when the amount of deviation re- 

mains the same, and 
(b) Non-comitant when the deviation changes notably, as the eyes 
are carried in different directions. 
A non-comitant deviation is usually of paralytic origin (paralytic 
squint or heterophoria). A comitant deviation is not usually due to 
paralysis, at least paralysis of the individual muscles, although it may 
develop out of such a paralysis. 

Deviations are also named according to the direction of the deviating 
eye. Thus — 

HETEROPHORIA. 

Both eyes fix when both are uncovered, but behind screen — 

Either eye turns in Esophoria. 

Either eye turns out Exophoria. 

E. eye turns up and L. down * R. Hyperphoria. 

L. eye turns up and R. down * L. Hyperphoria. 

* In some anomalous cases (see Section VI.), instead of one eye going up and the 
other down when screened, each eye goes up or each down. 



THE EXTRA-OCULAR MUSCLES 181 

SQUINT. 

When both eyes are uncovered one fixes and the other turns — 

In Esotropia (convergent squint, strabismus con- 

vergens), R. or L. 

Out Exotropia (divergent squint, strabismus di- 

vergens), R. or L. 

Up Hypertropia (upward squint, strabismus sur- 

sumvergens), R. or L. 
Down. . . .Hypotropia (downward squint, strabismus de- 
orsumvergens), R. or L. 
Behind screen — 

Either eye deviates in Esotropia (R. or L.). 

Either eye deviates out Exotropia (R. or L.). 

R. eye turns up and L. eye down * . . . R. Hypertropia or 

L. Hypotropia. 
L. eye turns up and R. eye down * . . . L. Hypertropia or 

R. Hypotropia. 
Esophoria, exophoria, esotropia, and exotropia are also known as 
lateral deviations (heterophoria, squint) ; hyperphoria, hypertropia, 
and hypotropia as vertical deviations. 

Mixed conditions, — e.g., combinations of a vertical with a lateral 
deviation, — are quite frequent. Especially common are the conditions 
prevailing in a developing squint (see Section IV.), in which hetero- 
phoria or orthophoria alternate with a true strabismus, or a squint for 
near is associated with heterophoria for distance, etc. Lastly, a patient, 
especially in paralytic cases, may show an esotropia if he looks in one 
direction and an exotropia if he looks in another ; or, he may have an 
esotropia for distance and an exotropia for near, as in a combined 
divergence- and convergence-paralysis (page 257) ; or he may have 
an esotropia if he fixes with one eye and exotropia if he fixes with the 
other. In the case last mentioned, one eye will deviate out when cov- 
ered, the other eye will deviate in (Graefe). (See Page 251, note.) 

Binocular Single Vision: Fusion. — As we have seen, in all but a 
small minority of cases binocular fixation is present whenever we look 
with both eyes. The image of the object looked at then is formed on 
each fovea. Yet, though there are the two images, we see, not two 
objects, but one (binocular single vision). Eor the mind by a peculiar 
process which we denominate fusion combines the two retinal images into 

* In some anomalous cases (see Section VI.) , instead of one eye going up and the 
other down when screened, each eye goes up or each down. 



182 THE EYE AND KEKVOUS SYSTEM 

a single visual impression. This binocular visual image differs some- 
what in character from the monocular visual image gained when we 
look with either eye by itself, being rather more distinct and also 
more solid-looking. In fact, vision with both eyes differs from vision 
with one eye in that with the former we get clear, and with the latter 
but imperfect, ideas of distance, depth, and relief. In other words, 
when we look with both eyes we have solid or stereoscopic vision. 

The mental faculty by which fusion is effected is called the fusion 
faculty. It acts, presumably, by means of a cerebral nerve-centre (fusion 
centre), the site of which, however, is altogether undetermined. Absence 
or non-development of the fusion faculty is thought to be an important 
factor — according to some, the prime factor — in the production of 
squint. 

Binocular Projection. — The visual impression produced by this fusion 
of the two retinal images is by the mind projected, or referred to a por- 
tion of space outside of the body, being thus regarded as a something 
apart from ourselves and occupying a certain place in the exterior 
world. The point to which we thus refer it is the point of intersection 
of our two visual lines. Unless the eyes abnormally deviate, this point 
corresponds to the actual situation of the object itself as determined by 
our other senses. Thus, when we look at an object one foot off, the eyes 
turn until their visual lines are both directed at the object, and hence 
intersect at the point where it is situated. Then each foveal image is 
projected out to this point of intersection, — i.e., to the place that the 
object itself actually occupies. 

Corresponding Points. — Not only does the mind fuse the two retinal 
images of any object looked at directly, so that that object appears 
single, but it fuses the images of a good many other objects as well. 
Thus, if we are fixing an object A with both eyes, A, which forms an 
image on each fovea, will appear single and will look to be straight in 
front of the eyes. Another object, B, situated to the right of A, will 
form its retinal image a certain distance, say 10° to the left of the 
fovea in the right eye. If now it is so situated as to form its retinal 
image 10° to the left of the fovea in the left eye too, it will appear 
single, the two outlying retinal images being fused in this case just 
as the two fovea! images were. In general, any object whose retinal 
images are formed equally far and in the same direction from the 
fovea in each eye — are formed as we say, on the corresponding points 
of the two retinas — will appear single. 

Diplopia. — -As we have just seen, when the images of the same object 
fall upon corresponding points, the object appears single. It is other- 



THE EXTRA-OCULAR MUSCLES 183 

wise when the images fall upon points not corresponding, — e.g., on the 
fovea in one eye and on any outlying portion of the retina in the other. 
Then, in general, we have double vision (diplopia), the mind receiving 
two visual impressions of the same object. In this case each eye pro- 
jects the image differently, — i.e., locates it in a different point in space. 
Physiological Diplopia. — In many cases this diplopia is physiological. 
Thus, in Eig. 3 the object A, at which both eyes are looking and which, 
therefore, forms its retinal images on the two fovese M and M' respec- 
tively, appears single, being projected by both eyes to its true place, A; 
but B, an object more remote than A and situated in the prolongation 
of the line MA, will appear double, for while it forms its image in the 
right eye on the fovea, M, it will form its image in the left eye on a 
point, N", to the right of the fovea. To the right eye it will appear 

Fig. 3. 



directly in line with A (behind it) ; to the left eye it will, according 
to the law of projection, appear to be to the left of A, and the more so 
the further N is to the right of M'. We shall have thus a condition of 
homonymous diplopia, — i.e., a condition in which the image seen by 
the left eye is to the left of that seen by the right eye. 

The object, C, lying nearer to the eyes than A and in the line, AM, 
will, to the right eye, seem to be in line with A (just in front of it) 
and to the left eye will seem to lie to the right of A, since C forms its 
image in the left eye at O to the left of the fovea. Such diplopia, in 
which the image seen by the left eye appears to the right of that seen 
by the right eye, is said to be heteronymous or crossed. This physio- 
logical diplopia, crossed for objects nearer than the object looked at and 



184 THE EYE AND KEKVOUS SYSTEM 

homonymous for more distant objects, although readily demonstrable, 
rarely, if ever, appears spontaneously and hardly ever occasions any 
confusion. 

Artificial Diplopia produced by Prisms. — Diplopia may also be pro- 
duced by prisms. Thus, suppose both eyes are looking at an object, A 
(Fig. 4) so that the image of that object falls on the fovea in each eye, 
and then a prism with the base directed inwards (towards the nose) is 
placed before the left eye. The prism will deflect the rays of light 
towards its base, so that the retinal image in the left eye will no longer 
fall on the fovea, M', but to the right of it, N. The eye, according to 
the law of projection, will regard this image as situated to the left of 
A, — i.e., the right eye fixing A with its fovea will see A in its true 
place, while the left eye will see a similar image, but dislocated to the 
left (at A'). There will then be a condition of homonymous diplopia. 

Fig. 4. 




So also, a prism placed base out before one eye will produce crossed 
diplopia. A prism placed base up or base down will make the corre- 
sponding image appear below or above the other, — i.e., will cause a 
vertical diplopia. 

Pathological Diplopia. — Diplopia is also caused by a deviation of the 
eyes. That is, if the right eye (E. Fig. 5) is fixing the object A, but 
the left eye, instead of fixing it, is turned too far inwards, the retinal 
image of A in this eye will fall not on the fovea W, but to the inner or 
right side of it (N). Now, the mind really does not know how the left 
eye is pointing; in fact, it seems to regard both eyes as looking in the 
same direction; hence, an object whose image is on the fovea of the 
left eye will be projected out to A, the direction in which the mind con- 



THE EXTEA-OCULAK MUSCLES 185 

ceives this eye to be jx)inting (page 178), and an object whose reti- 
nal image is at X, to the right of the fovea, will be regarded as located 
correspondingly far to the left of A. Such a patient, therefore, sees 
two images of A — one seen by the right eye, situated in its true place 
straight ahead ; the other seen by the left eye, situated to the left (A') — 
i.e., there is here an homonymous diplopia. If the left eye had been 
turned out there would have been a crossed diplopia ; if up or down, 
a vertical diplopia. 

Overcoming Diplopia. — Diplopia produced by prisms, or diplopia pro- 
duced by pathological deviation, may be overcome if the eye is turned 
so that the image in either case falls upon the fovea. Thus, the homony- 
mous diplopia shown in Eig. 4 and in Fig. 5 will be abolished if the left 
eye turns out as shown by the arrow, for, turning the eye out will turn 

Fig. 5. 




the fovea M' in, and when M' has gone in far enough the retinal image 
of A will fall upon it; then the retinal image will fall on the fovea 
in each eye, and there will be single vision (See Page 181). 

The ability to overcome diplopia depends partly upon the readiness 
with which the various movements required for the purpose can be exe- 
cuted, partly upon the development of the fusion faculty ( See Page 182 ) . 
A man, however well developed and well regulated his eye muscles and 
the nerve-centres that co-ordinate them may be, will not unite double 
images well if his fusion faculty is imperfect. Ill development or 
temporary abeyance of the fusion faculty probably accounts in part for 
the fact that in some cases of neurasthenia, hysteria, and organic brain 
disease, diplopia is so easily produced and when produced is so difficult 



186 



THE EYE AND NEKVOUS SYSTEM 



to overcome. In such cases the mere act of placing a red glass or a 
1° prism before one eye suffices to evoke an insuperable diplopia. 
Again, there are some persons who never by any adjustment of the eyes 
or with any prism get fusion of the double images, — in whom, for 
instance, a prism of 6°, base out, produces a crossed, and a prism of 
5°, base out, a homonymous diplopia, but no prism produces single 
vision. This condition of so-called antipathy to single vision occurs in 
squint, particularly after operation, and is probably attributable to non- 
development of the fusion faculty. 

Varieties of Diplopia. — The foregoing facts, together with the nomen- 
clature of the different varieties of diplopia are succinctly shown in the 
following table : 

TABLE OF DIPLOPIA.* 



NAME OF DIPLOPIA 


CHARACTER 


CAUSED BY 


CORRECTED BY 




Image of R. 
eye as com- 
pared with 
that of L. is 


(1) A natural 
deviation of 


(2) Artifici- 
ally by a 
prism placed 
base. 


(1) Turning. 


(2) Prism 
placed with 
base. 


H " 
Eh 

< 

< 

O 

pa 
w 

> 


Homony- 
mous. 

Heterony- 
mous. 
^(Crossed.) 

rRight. 
Left. 


On the right. 
On the left. 

Below. 
Above. 


Either eye in- 
ward (esopho- 
ria, esotropia). 

Either eye 
outward ( ex- 
ophoria, exo- 
tropia). 

R. eye up or 
L. eye down 
(R. hyperpho- 
ria, R. hyper- 
tropia, L. hy- 
potropia. 

R. eye down or 
L. eye up (L. 
hyperphoria, 
L. hypertro- 
pia, R hypo- 
tropia). 


In, before 
either eye. 

Out, before 
either eye. 

Up before R. 
eye, down 
before L. eye. 

Down before 
R. eye, up 
before L. eye. 


Both eyes out- 
ward. (Di- 
vergence. ) 

Both eyes in- 
ward. ( Con- 
vergence. ) 

R. eye down 
and L. eye up 
( L. supra ver- 
gence). 

R. eye up and 
L. eye down 
( R. supraver- 
gence). 


Out before 
either eye. 

In before 
either eye. 

Down be- 
fore R. eye 
or up be- 
fore L. eye. 

Up before 
R. eye or 

down before 
L. eye. 



In conditions producing vertical diplopia, especially when the eyes 
are directed down, one image (usually the one that should be higher) 
often seems to be on a level with the other, but back of it. This is due 
partly to the character of the background upon which the images are 
projected, partly to other factors (M. Sachs, Frolich). 



* Modified from the author's article in De Schweinitz and Randall's 
Text-Book of the Eye, Ear, Nose, and Throat." 



American 



THE EXTKA-OCULAE MUSCLES 187 

Character of the Images in Diplopia. — In diplopia the image seen by 
the fixing eye is usually sharp and clear because that eye sees it with 
the fovea, the portion of the retina having the most distinct vision. 
This image is hence called the real image. The other, or false, image, 
projected by the non-fixing eye, looks shadowy and indistinct, because 
it is seen by an extrafoveal portion of the retina. 

Binocular Fixation and Binocular Single Vision compared. — De- 
rangements of binocular fixation cause the various deviations of the eye 
(squint or heterophoria). Such a derangement is proved to exist if 
the deviation can be made out objectively, — i.e., if there is a deflection 
of the eyes which the observer can discover on inspection of the patient. 

On the other hand, disturbance of binocular single vision is proved 
to exist if the patient himself has the subjective sensation of diplopia. 

Upon these two facts depend all of our tests, which are hence classed 
as objective when they show disturbance in binocular fixation which the 
observer can detect, and subjective when they depend directly or indi- 
rectly upon the demonstration of diplopia. 

The two conditions — disturbance of binocular fixation and disturb- 
ance of binocular single vision — usually occur together and are equally 
marked. In this case the objective and subjective tests can both be 
employed and will, moreover, agree with each other. In two classes of 
cases, however, this is not so : 

I. In most cases of old squint binocular vision is absent — that is, 
when both eyes are open the image formed on the retina of the squint- 
ing eye is mentally suppressed so that the patient sees with the other 
eye only. When the squinting eye alone is used for seeing, this sup- 
pression does not occur ; hence if a red glass is placed before the squint- 
ing eye and the patient looks at a light with his right eye shut he will 
see a red light, while if he either has both eyes open or has the left eye 
shut he will see simply a white light. In these cases, therefore, the 
patient cannot be made to recognize diplopia ; hence, all the subjective 
tests which in one way or another depend upon the recognition of diplo- 
pia, fail, and we have to depend entirely on the objective tests (those 
for binocular fixation). 

II. In many cases of squint, and particularly of cured squint, both 
subjective and objective tests are applicable, but are discrepant because 
the patient projects or locates objects in space erroneously with his 
squinting eye. Thus, we may have a condition in which the eye is 
obviously turned in, and yet there is either a crossed diplopia, or, if 
there is a homonymous diplopia it does not correspond in amount to 
the degree of deviation (incongruity of the double images). 



188 THE EYE AND NEKVOUS SYSTEM 

Muscles of the Eye. — The muscles moving the eyeball and eyelids 
are the internal, external, superior, and inferior recti, the superior and 
inferior obliques, and the levator palpebral 

The four recti and the levator arise from the lesser and greater wing 
of the sphenoid and from the dense fibrous membrane filling in the 
sphenoidal fissure. Their combined origins form a tendinous ring 
which surrounds the optic foramen and that portion of the sphenoidal 
fissure giving passage to the oculomotor and abducens nerves. These 
five muscles pass forward, diverging as they go, so as to form a hollow 
cone (muscle cone), which includes the second, third, and sixth nerves, 
and the ciliary ganglion. 

The superior rectus lies underneath the levator, to which it is con- 
nected quite firmly by bands of fascia. The other muscles at first run 
quite close to the walls of the orbit, but somewhat in front of the middle 
of their course the four recti turn in toward the eyeball, and piercing 
the orbital fat and Tenon's capsule, are inserted into the sclera around 
the cornea. The internal rectus is inserted 5.5 millimetres, the inferior 
6.5 millimetres, the external 7 millimetres, and the superior 7.5 to 8 
millimetres from the corneal margin. The levator passes on to be 
inserted into the tarsus of the upper lid. 

The superior oblique arises from the body of the sphenoid, runs for- 
ward, keeping close to the upper wall of the orbit, and at the upper 
inner margin of the orbit passes through a tensinous ring (the tro- 
chlea), from which it is reflected obliquely backward and outward so as 
to pass beneath the tendon of the superior reitus. It pierces Tenon's 
capsule, and is inserted into the upper outer portion of the eye back of 
the equator. 

The inferior oblique arises from the inner lower wall of the orbit just 
back of the nasal duct, passes obliquely up and out beneath the inferior 
rectus, and is inserted into the lower outer part of the eyeball behind 
the equator. 

Mention has been made of Tenon's capsule. This is a spherical 
capsule of connective tissue surrounding the whole eye except the cornea 
and a little rim of sclera around it in front, and the optic nerve and a 
little annular area of sclera about it behind. It is thus like a broad 
convex ring surrounding the eye like a sling. The anterior margin of 
this ring is fused with the conjunctiva, and both conjunctiva and cap- 
sule are attached firmly to the sclera round the cornea. The posterior 
margin of the capsular ring is also attached to the sclera, so that the 
space between the eyeball and Tenon's capsule is closed in, both anteri- 
orly and posteriorly. This space, called Tenon's space, is traversed by 



THE EXTKA-OCULAK MUSCLES 189 

connective tissue bands joining the capsule to the eyeball. Tenon's 
space is held to be the analogue of a joint cavity, although it is filled 
neither with synovia nor with lymph. This analogy must not be pushed 
too far, for while the eye does, in fact, turn in the orbital tissue as in a 
socket, it is probable that as Tenon's capsule must, to a certain extent, 
move with the eye to which it is attached, the real socket of the eyeball 
lies in the orbital fat outside of the capsule (Motais, cited by Maddox). 

In considering the action of the muscles, we have to take into ac- 
count not only the primary insertion (to the eyeball), but also the 
secondary insertion (to the orbital walls and eyelids). 

The primary insertion of each muscle is by a broad, flat tendon, 
implanted into the sclera beneath the conjunctiva and Tenon's cap- 
sule. This insertion is reinforced by a fibrous band on each side con- 
nected with the sclera and extending about one to one and one-half milli- 
metres in front of the insertion proper. These adminicula, as they are 
called, like the insertion itself, lie beneath Tenon's capsule, — i.e., within 
the capsular cavity. Other bands are given off from the tendon just 
before it enters the capsule which attach the tendon to the outer surface 
of the latter. 

The secondary insertions of the tendons are : 

I. Check ligaments or firm bands of fascia extending from the 
anterior portion of the muscles to the orbital walls. 

II. The palpebral bands extending to the septum orbitale and the 
lids. 

The check ligament of the internal rectus unites with that of the 
inferior rectus and is inserted into the lachrymal bone behind the lach- 
rymal sac. 

The external rectus has two check ligaments, — one below, the other 
above, — both attached to the outer wall of the orbit near its margin. 

The superior rectus and levator palpebral have two check ligaments. 
The outer, broad and firm, fuses with the upper check ligament of the 
externus and forms a firm septum supporting the lachrymal gland, and 
is attached to the upper wall of the orbit near its margin. The inner 
check ligament is attached to the upper, inner part of the orbit as far 
forward as the trochlea. 

The superior oblique has two check ligaments — one, the posterior, 
running from the belly of the muscle to the trochlea ; the other, the 
anterior, running from the trochlea backward upon the reflected tendon 
to which it is attached. 

The inferior oblique has no proper check ligament. It is attached 
by very strong fibrous bands to the inferior rectus, the fascia here being 



190 THE EYE AND NERVOUS SYSTEM 

so dense and strong as to act like a sling supporting the eyeball (hence, 
called by some the suspensory ligament of the eye). 

The action of a check ligament is twofold : first, it restricts the action 
of the corresponding muscle so as to limit very considerably the rota- 
tion the latter would otherwise produce; second, it prevents the recti 
muscles from retracting the eyeball into the orbit. In fact, the eyeball, 
because it is held by the various check ligaments, and also because it 
is pulled in opposite directions by the obliques (which tend to draw it 
forward), and by the recti (which tend to draw it backward), is kept 
slung in one position, so that, instead of advancing or receding, it simply 
rotates in its socket. 

The place of a check ligament for the inferior oblique is taken by 
the anterior ligament of the superior oblique which becomes tense in 
proportion as the inferior oblique contracts and the superior oblique 
relaxes. 

The palpebral bands are the superior, running to the tarsus of the 
upper lid from the fascia connecting the superior rectus with the levator 
palpebral, and the inferior, running to the tarsus of the lower lid from 
the fascia between the inferior rectus and the inferior oblique. When 
the superior rectus contracts, the superior palpebral band is pulled upon 
and the upper lid is elevated so that lid and eye rise together. So also 
when the inferior rectus contracts, the lower lid is pulled downwards 
by the inferior palpebral band. 

Actions of the Ocular Muscles. — The actions of the eye muscles may be 
divided into main and subsidiary. 

1. The main actions are as follows: The external rectus abducts 
the eye, — i.e., moves it straight outwards or towards the temple. 

The internal rectus adducts the eye, — i.e., moves it straight in or 
towards the nose. 

The superior rectus elevates the eye. As the muscle runs from 
within outward, its elevating action will be greatest with the eye ab- 
ducted, because then it will be in line with the anteroposterior axis of 
the eye and will thus work to the greatest advantage. On the other 
hand, when the eye is adducted the muscle will be nearly at right angles 
to the anteroposterior axis of the eye, and will hence have little or no 
action as an elevator. 

The inferior oblique acts to pull the back part of the eye down, and 
hence throws the front part up. Like the superior rectus, therefore, it 
is an elevator of the eye. As it runs quite obliquely backward and out- 
ward its elevating action will be greatest wdien the eye is adducted, for 
then the muscle will be nearly in line with the anteroposterior axis o£ 



THE EXTEA-OCULAE MUSCLES 191 

the eye. On the other hand, when the eye is abducted its elevating action 
will be practically nil. 

The inferior rectus depresses the eye, and, like the superior rectus 
to which it is nearly parallel, it will depress the eye most energetically 
when the latter is abducted, and little or not at all when it is adducted. 

The superior oblique elevates the back part of the eye, and hence 
throws the front part down like the inferior rectus ; therefore, it is a 
depressor, but, owing to the oblique way in which it runs, it depresses 
the eye the most when the latter is adducted, and little or not at all 
when it is abducted. 

The levator lifts the upper lid. 

2. The subsidiary actions of the eye are as follows : 

I. Lateral Action of Vertical Muscles. — The superior and inferior 
recti, owing to the obliquity of their course, adduct the eye, and the 
more so when the eye is already adducted. 

The two obliques, for a similar reason, abduct the eye, and the more 
so the more the eye is already abducted. 

II. Torsion Movements. — The superior rectus and the superior 
oblique intort the eye, — i.e., rotate the top of its vertical meridian 
inward, or towards the nose. 

The inferior oblique and inferior rectus extort the eye, — i.e., rotate 
its vertical meridian outward. 

The intorting action of the superior rectus and the extorting action of 
the inferior rectus are most pronounced when the eye is adducted ; the 
intorting and extorting action of the obliques is most pronounced when 
the eye is abducted. 

III. Action of Eye-Muscles on Lids. — The superior rectus by virtue 
of its palpebral band elevates the upper lid, and the inferior rectus 
depresses the lower lid. 

Monocular Movements. — From what has just been said it is evi- 
dent that the eye is adducted mainly by the internal rectus, assisted 
towards the end of its course by the superior and inferior recti. 

It is abducted by the external rectus, reinforced towards the end of 
its course by the two obliques. 

It is elevated by the superior rectus and inferior oblique. The supe- 
rior rectus acting alone would carry it up and a little in ; the inferior 
oblique acting alone would carry it up and a little out ; the two muscles 
acting together would carry it straight up. It is quite certain from 
the results, both of physiological experiment and the data derived from 
cases of paralysis, that when we look up both muscles do always act 
together. When we look straight up, both act quite strongly as elevators, 



192 THE EYE AND NEKVOUS SYSTEM 

although the superior rectus, having the less oblique course, is rather 
more efficient than the inferior oblique. When we look up and out, 
although both muscles still act, the superior rectus is the only one that 
lifts the eye, the superior oblique simply tending to abduct it still more 
and thus reinforce the action of the external rectus. Per contra, when 
we look up and in, the inferior oblique is the main agent in lifting 
the eye, the superior rectus simply acting to carry the eye inward and 
so reinforce the action of the internal rectus. 

In all movements of elevation of the eye, whether straight up, up 
and in, or up and out, the upper lid is simultaneously elevated by the 
levator and the superior rectus. 

The eye is depressed by the inferior rectus and superior oblique. 
The inferior rectus acting alone would carry it down and a little in; 
the superior oblique down and a little out. As a matter of fact, both 
muscles always act together and the eye is carried straight down through 
their combined action, the inferior rectus being rather the more effective 
of the two. When we look down and out the inferior rectus carries the 
eye down and the superior oblique carries it out, reinforcing the action 
of the externus. When we look down and in the superior oblique carries 
the eye down, the inferior rectus acting simply to carry the eye in and 
reinforce the action of the internal rectus. 

In all movements of depression of the eye, whether straight down, 
or down and in, or down and out, the lower lid is simultaneously de- 
pressed by the inferior rectus. 

Movements of the Lids. — The upper lid is raised by the levator, as- 
sisted by an unstriated muscle, the superior muscle of Miiller, which is 
supplied by the sympathetic. Moreover, when the eye is elevated, so 
that the superior rectus is brought into play, this muscle also takes part 
in raising the lid. 

The lower lid is depressed, partly by its own weight, partly by the 
contraction of the unstriated inferior muscle of Miiller, which is sup- 
plied by the sympathetic. Moreover, when the eye is depressed, so that 
the inferior rectus is brought into action, this muscle also takes part in 
depressing the lid (See Chapter VII). 

Field of Fixation. — By the action of the several muscles, either single 
or combined, the eye is able to make excursions in every direction — 
up, down, in, out, and obliquely. In this way, without moving his head, 
one can fix in succession all the objects occupying a large portion of 
the space about him. By thus moving the eye as far as possible in 
every direction, we delimit what is called the field of fixation. (See 
Section on Tests.) 



THE EXTKA-OCULAK MUSCLES 193 

Position of Equilibrium, or Primary Position. — When all the muscles 
tHat move the eve are at rest, or at least when no one of them is con- 
tracting more than any of its fellows, the eye assumes its position of 
equilibrium, or what is called the primary position. This position, in 
the great majority of cases, is such that the line of sight is perpendicular 
to the line connecting the centres of rotation of the two eyes, and lies 
in or slightly below the horizontal plane, — i.e., the eye looks right ahead 
and on the level or a little below it. 

Field of Action of the Muscles. — As we have just seen, the external 
rectus acts to carry the eye directly outward. TvTien the eye is turned 
in, this muscle relaxes, and the only effect it produces is that due to the 
passive traction exerted by its tendon. Thus, the external rectus acts 
only in the outer half of the field of fixation. This outer half of the 
field of fixation we may then denominate the field of action of the 
external rectus. Similarly the field of action of the superior rectus 
will be the upper half of the field of fixation, and if we have regard 
only to the elevating action of the superior rectus, its field of action 
will be confined mainly to the upper outer quadrant of the field of 
fixation. 

Erom a similar consideration it is evident that each one of the six 
muscles moving the eye has its special field of action, as shown in the 
following table: 

Muscle. Special Field of Action. 

External rectus. Outer half of field of fixation. 

Internal rectus. Inner half of field of fixation. 

Superior rectus (as an elevator). Upper and outer quadrant of field of 

fixation. 
Inferior oblique (as an elevator). Upper and inner quadrant of field of 

fixation. 
Inferior rectus ( as a depressor ) . Lower and outer quadrant of field of 

fixation. 
Superior oblique (as a depressor). Lower and inner quadrant of field of 

fixation. 

Binocular Movements. — The two eyes when acting together can- 
not move independently of each other. The whole purpose of the neuro- 
muscular apparatus of the eyes is to secure binocular fixation. Hence, 
movements which will interfere with binocular fixation are not ordi- 
narily performed, and, more than that, cannot be performed except to 
a very limited degree. The movements actually possible to the two eye? 
working together are as follows : 

I. Parallel Movements. — This class of movements is employed when 
we carry the eye from one distant object to another. In this case the 
13 



194 THE EYE AND KEKVOUS SYSTEM 

two visual lines remain parallel with each other, both moving equally 
far and equally fast to the right, to the left, up, down, and obliquely. 

' An analysis of the statements made regarding the actions of the 
muscles for moving each eye individually (see Section on Monocular 
Movements) will show that the twelve muscles moving both eyes may 
be classed as follows: 

A. LATERAL ROTATORS. 

Producing lateral rotation of both eyes — lateriversion* 

(a) Right rotators (acting when the (b) Left rotators (acting when the eyes 

eyes are directed to the right). are directed to the left). 

Producing rotation to the right (dextro- Producing rotation to the left {levo- 
version ) . version ) . 

R. External rectus. R. Internal rectus. 

L. Internal rectus. L. External rectus. 

B. ELEVATORS. 

Producing elevation of both eyes ( sursumversion ) . 
(a) Right-handed elevators (acting (6) Left-handed elevators (acting 
mainly when the eyes are di- mainly when the eyes are di- 

rected to the right). rected to the left). 

R. Superior rectus. R. Inferior oblique. 

L. Inferior oblique. L. Superior rectus. 

C. DEPRESSORS. 

Producing depression of both eyes ( deorsumversion ) . 
(a) Right-handed depressors (acting (b) Left-handed depressors (acting 
mainly when the eyes are directed mainly when the eyes are directed 

to the right). to the left). 

R. Inferior rectus. R. Superior oblique. 

L. Superior oblique. L. Inferior rectus. 

It will be seen that the twelve muscles are divided into three groups, 
and each group comprises two pairs, the muscles of one pair acting when 
the eyes are directed towards the right, the muscles of the other pair 
acting when the eyes are directed to the left. 

This makes six pairs of muscles — one muscle of each pair being in 
the right eye, the other in the left. The two muscles constituting a 
pair are called associates. Two associates acting together will move 
the right eye and the left eye respectively in the same direction and to 
the same extent. Thus, the right inferior rectus moves the right eye 
down, to the left, and rotates its vertical meridian to the right. Its 

* In lateriversion the movements of abduction made by one eye and of adduction 
made by the other are called latero-abduction and latero-adduction, respectively 
( Schapringer ) . They are to be distinguished from divergence-abduction and con- 
vergence-adduction (pages 195, 196). 



THE EXTKA-OCULAK MUSCLES 195 

associate, the left superior oblique, moves the left eye down, to the left, 
and rotates its vertical meridian to the right. 

The precision with which parallel movements are performed is re- 
markable. Under ordinary circumstances, parallelism is maintained 
up to the limits of excursion of the eyes in any given direction. If this 
is not the case — if one eye for any reason fails to keep pace with the 
other in advancing in any particular direction — diplopia ensues. The 
point where such diplopia begins indicates the limits of binocular single 
vision^ in that special direction of the gaze, and by determining these 
limits for all directions of the gaze — right, left, up, down, and obliquely 
— we can map out what is called the field of binocular vision. 

As we shall see later, any marked restriction of the field of binocular 
single vision in some one direction of the gaze, indicates an insufficiency 
or paralysis of one of the ocular muscles. 

There appears to be a distinct nerve-centre or tract governing each 
one of the different parallel movements of the eyes, — i.e., there is one 
centre governing the movements to the right, one governing the move- 
ments to the left, one governing upward movements, etc. The centres 
for the initiation of such movements {centres of voluntary movement) 
are apparently in the frontal lobe (Russell, Mott and Schafer, Sherring- 
ton, Topolanski). In addition to these there are posterior cortical cen- 
tres which are undoubtedly situated in the angular gyrus (Bernheimer). 
These are reflex centres, — i.e., designed for movements set up in a reflex 
way by visual impressions. Stimulation of either the anterior or the 
posterior cortical centres on the right side produces a movement of both 
eyes to the left (or up and to the left or down and to the left), and, if 
very strong, also produces inhibition of the movement to the right. If the 
stimulus starts from the angular gyrus, it crosses over just beneath the 
aqueduct to reach the subcortical centres which are found in and about 
the nerve nuclei of the opposite side. The thalami and corpora quadri- 
gemina take no part in the production of parallel movements (Topo- 
lanski). 

II. Movements of Convergence. — When we pass from a distant object 
to one nearer we have to converge the eyes. We do this by means of 
a simultaneous and equal contraction of the two internal recti, produc- 
ing an equal movement inward {convergence-adduction) of each eye. 
This movement of convergence may be combined with a lateral or verti- 
cal parallel movement, as when we look at a near object that is situated 
to the right or left, or is situated above or below the horizontal plane. 

The centre for the initiation of convergence-movements (voluntary 
movements of convergence) is probably in the frontal lobe (according 



196 THE EYE AND KEKVOUS SYSTEM 

to two experiments of Sherrington's, near the angle of the precentral 
fissure). Sherrington also found that inhibition of convergence could 
be produced by cortical stimulation. The posterior cortical (reflex) 
centres are said by Bechterew to be in the occipital lobe behind the 
junction of the Sylvian with the first temporal fissure and in the angu- 
lar gyrus. The subcortical centres, according to Bechterew, are, first, 
one in the third-nerve nucleus, probably connected with the frontal 
centre; and, second, one in the corpora quadrigemina, probably con- 
nected with the posterior cortical centres. According to Bernheimer, 
the first of these subcortical centres would comprise the partial nuclei 
for the internal rectus on either side, the commissural fibres connect- 
ing the right and left partial nuclei, and the fibres running from each 
partial nucleus to the internus of the same side (the crossed fibres 
running to the internus of the other side subserving not convergence 
but latero-adduction). (Compare Page 13). 

III. Movements of Divergence. — When our eyes are converged on a 
near object and we then look at an object more remote, the lines of 
sight of the two eyes separate, or, as we say, the eyes diverge. This 
movement of divergence is equal and simultaneous in the two eyes, each 
one moving out equally far and fast. According to some, the movement 
is produced by the relaxation of the two internal recti (passive diver- 
gence) ; according to others, by the contraction of the two external recti 
(active divergence, divergence-abduction). In all probability, it is 
really due to these two actions combined. According to Bechterew, the 
centre for the initiation of divergence (voluntary divergence move- 
ments) lies in the frontal lobe in front of the middle of the precentral 
fissure. The posterior (reflex) centre is in the angular gyrus. The 
subcortical centre is in the abducens nuclei. 

IV. Divergence in the Vertical Plane. — To a very slight extent the 
lines of sight of the two eyes can separate in a vertical direction. The 
movement is doubtless equal and simultaneous in the two eyes, one eye 
rising while the other descends. It is called supravergence, and is 
denoted as right or left, according as the right or left eye is the higher. 

Tests. — In testing for derangements of the ocular muscles and ocular 
movements we have to determine : 

I. Whether each eye by itself can perform fixation. (Tests of mon- 
ocular fixation.) 

II. Whether, when in the primary position, both eyes always or 
usually perform binocular fixation or whether they deviate. (Tests 
of binocular fixation. ) 

III. Whether, the eyes being in the primary position, the patient 



THE EXTKA-OCULAK MUSCLES 197 

has or has not binocular vision, or, if he has, whether he has binocular 
single vision, or else either diplopia or a tendency to diplopia. (Tests 
for binocular vision ; diplopia tests. ) 

IV. Whether each eye by itself can move freely in all directions. 
(Tests of monocular movement; determination of field of fixation.) 

V. Whether the two eyes working together can perform their regu- 
lar co-ordinated movements, and particularly whether they can perform : 

(a) Parallel movements. (Determination of binocular excursions 
and of the field of binocular single vision.) 

(&) Movements of convergence. (Tests of convergence.) 

(c) Movements of divergence. (Tests of divergence.) 

A great many tests are utilized, but only the more important will 
be mentioned here; those given, however, being sufficient in all ordi- 
nary cases to make an adequate diagnosis. 

In making all the tests the patient's head must be kept erect and 
motionless, and the face * must not be turned either to the right or to the 
left. 

The test-object used should be sharply defined, be just large enough, 
be distinctly seen, offer a marked contrast to its surroundings, and be 
in the same plane as the latter. An excellent test-object is a white target 
20 to 30 centimetres in diameter, with a black bull's-eye. The latter, 
if the tests are made for distance (fifteen or twenty feet), should be 
8 millimetres in width, and the target itself should then be set flat on a 
large dead-black surface. If the tests are made at near points the bull's- 
eye should be 1 or at most 2 millimetres wide. Another good test- 
object, indispensable for some of the tests, is a small brilliant light set 
against a large, flat, dead-black background. For near tests this may 
conveniently take the form of a minute electric light set in the centre of 
a metal disk which is covered with black cloth. 

In most of our tests we measure the amount of deviation present by 
means of prisms. In so doing we must recollect that prisms are usually 
numbered according to their refracting angle, and that the amount of 
deviation that they produce — and consequently the . amount of deviation 
that they measure — is only one-half or a little more than one-half of 
this angle. Thus a deviation that is neutralized by a prism of 10° is 
actually one of only 5° of arc, a deviation neutralized by a prism of 20° 
is 11° of arc, etc. 

I. Tests of Monocular Fixation. — We make the patient cover one e} r e 
and then direct his other eye sharply at some well defined object, like 



* Except when determining the field of binocular single vision for distance. 



198 THE EYE AND NEKVOUS SYSTEM 

a pencil or a light, held straight in front of him. Inspection then will 
show, approximately at least, whether his fixation is central or eccentric, 
or whether, as in cases of a high grade amblyopia, he cannot fix at all, 
but allows his eye to wander aimlessly, getting mere glimpses of the 
object shown him. 

II. Tests of Binocular Fixation. — Of the several tests for binocular 
fixation, by far the best and most reliable is the screen test, and this 
alone will be described here. In performing this we have the patient 
look at an object fifteen feet or more from the eyes. 

The patient is directed to look sharply at the object (bull's-eye or 
light), and then the card is placed before the right eye and passed 
quickly to and fro from one eye to the other. 

If there is orthophoria, neither eye will deviate when covered, and 
each consequently will remain steady when the screen is removed. If 
there is squint or heterophoria of more than a degree or two, each eye 
when covered will deviate perceptibly, and when uncovered will turn 
back into the fixing position. Thus, if there is either an esophoria or 
convergent squint each eye in turn will deviate in or towards the nose 
when covered, and will swing out again when uncovered. 

Whether the tico eyes deviate to the same amount or not behind the 
screen will depend upon which has the stronger muscles. Equally, in 
the case of paralysis of the right eye with normal musculature in the 
left, and in the case of spasm of the left eye with normal musculature in 
the right, the deviation of the right eye behind the screen will be less 
than that of the left. (Compare remarks under Diagnosis of Paralysis, 
Section II., and Symptoms of Spasm, Section III.) The same thing 
occurs in certain cases of squint. (See Note to Table of Periodic 
Heterophoria and Squint, Section IV.) 

The amount of the deviation can be measured accurately by means 
of the prism that abolishes it. Thus, if the eye is deviated out behind 
the screen, showing that there is an exophoria or a divergent squint, 
the amount of the deviation may be measured by the prism, placed base 
in before the eye, that stops the movement altogether. Usually, in 
measuring the deviation in this way we add prisms until the movement 
is not simply abolished, but is converted into a movement in the opposite 
direction. The first prism that causes this over-correction, less 2°, will 
represent pretty closely the amount of the deviation. 

Having ascertained in this way that a deviation is present and how 
much it is, we next inquire whether this deviation is a squint or a 
heterophoria. 

To determine this we make the patient look fixedly at the test-object 



THE EXTRA-OCULAR MUSCLES 199 

with both, eyes open, and then alternately cover and uncover one eye, — 
say the right, — leaving the other all the time uncovered (method of 
binocular uncovering). By thus covering the right eye we compel the 
left eye to fix, if it is not already doing so, and we allow the right eye to 
deviate if it has any tendency to do so if it is not already deviating. 
In this case three conditions may obtain : 

(a) There may be heterophoria. In this event the left eye which 
is fixing already will continue fixing when the right eye is covered, 
and the latter which was fixing before being covered will not deviate. 
Then, when the cover is taken off again, the right eye will swing back 
into place and the left will still remain fixing, — i.e., both on applying 
and on removing the screen but one eye will move, and that the right, 

(b) There may be a squint of the right eye. In this case the left 
eye which was fixing before the right was covered will continue fixing 
afterwards, and the right eye which was deviating before it was cov- 
ered will remain deviating afterwards. Then, on uncovering, the left 
eye will still remain fixing and the right will still remain deviating, — 
i.e., both on applying and on removing the screen neither eye moves, pro- 
vided the screen is put over the squinting eye. 

(c) There may be a squint of the left eye. Then the left eye, which 
was deviating before the right eye was covered, will now have to move 
into place in order to fix. As it moves into place, the right eye, which 
is behind the screen, will move out of place. Then, when the screen is 
removed, the right eye which has thus become deviated, will move back 
into the fixing position, since it is the right eye which ordinarily fixes 
when both eyes are open, and the left eye will move out of the fixing 
position and back to its ordinary position of deviation, — i.e., both on 
applying and on removing the screen both eyes will move, provided in 
the case of a squint the screen is put before the fixing eye. 

If repeated applications of the test show that sometimes the right 
eye squints, sometimes the left, then we are evidently dealing with 
an alternating strabismus. 

III. Tests of Binocular Vision. — To determine whether the patient 
has binocular vision or not, and, in case he has binocular vision, whether 
there is diplopia or a tendency to diplopia, we employ a large number 
of tests. 

As has already been said, the tests of binocular vision do not agree 
with those for binocular fixation, — e.g., with the screen test, — if there 
is any incongruity of the double images (See Page 187). 

Again, if there is suppression of the image in one eye, such as 
regularly takes place in squint, all the tests for binocular vision will 



200 THE EYE AND NEKVOUS SYSTEM 

fail. Per contra, the failure of these tests in any given case argues the 
existence of suppression. 

Only the more important tests will be given here : 

1. Parallax Test. — As we are passing the card from eye to eye in 
making the screen test, we ask the patient whether the object he is 
looking at seems to move or not. If it does, it means that he actually 
sees the object double, only instead of seeing the two images simultane- 
ously he sees one after the other. Thus, if on uncovering the right eye 
the object seems to move to the right it means that the right-eye image 
is to the right of the left, — i.e., he has homonymous diplopia. If, under 
the same conditions, the object had seemed to move to the left he would 
really have had crossed diplopia. If it had moved down he would have 
had a right diplopia ; if up, a left diplopia. 

In order to be sure that the patient is actually fixing the object and 
not looking beyond it, so that he sees it projected against a background 
more remote, the test-object should be on the same plane as the objects 
immediately surrounding it. Hence, as already stated, we make the 
patient look at a small spot or light set on a large blank surface. 

The amount of the parallax, whether homonymous, crossed, right, 
or left, as the case may be, may be measured accurately by the prism 
which, placed before the eyes, will cause its abolition. Thus, an homony- 
mous parallax which is completely abolished by a prism of 15° base 
out, indicates an esophoria of 15°, etc. 

2. Red Glass. — If there is much tendency to diplopia the mere inter- 
position of a red glass before one eye will evoke it. In this case the 
test object should be a light, of which, then, two images will be seen — 
one red, the other white. This proves that the patient has binocular 
vision with diplopia. If the red glass is placed before the right eye 
the red image will be on the right hand if the diplopia is homonymous ; 
on the left hand if the diplopia is crossed ; below if there is right diplo- 
pia, and above if there is left diplopia. 

If, with the red glass before one eye, the patient sees a single light 
which is white tinged with red, he surely has no diplopia, and has binoc- 
ular single vision. 

If he sees a single light which is pure white or pure red, he is sup- 
pressing the image in one eye (page 178). In this case he has no 
diplopia, and has only monocular vision. 

The amount of deflection, and hence the amount of deviation, may 
be determined either by ascertaining the linear distance between the 
double images (allowance being made for the distance of the object 
from the patient), or by finding out what prism will be required to over- 



THE EXTKA-OCULAK MUSCLES 201 

come the diplopia. The latter method, however, usually gives an insuffi- 
cient measure of the amount of the diplopia. The real diplopia, for 
example, may be 20° and yet be apparently nullified by a prism of 
18°, because such a prism brings the images so close together that the 
patient then unites them himself without further aid. 

In testing for diplopia in this way we ask the patient not only how 
the double images are placed with regard to each other and how far 
apart they are, but also whether both are upright or not. If one of 
the images is tilted, either to the right or to the left, it indicates that 
the corresponding eye is tilted and has its vertical meridian rotated in 
the opposite direction. Thus, if the candle seen by the right eye tilts 
to the right, it means that the right eye itself is tilted to the left or has a 
pathological intorsion. 

3. Maddox Rod. — The Maddox rod consists of one or more little 
cylinders of glass set in a frame (Eig. 6). If the instrument is held 

Fig. 6. 




as shown in the figure and placed before the right eye, the patient, 
looking at the light, will see with this eye the light transformed into a 
luminous vertical streak. If he has neither esophoria nor exophoria 
this streak will appear to run through the flame which in its unaltered 
form he sees with the left eye. If he has esophoria it will appear to the 
right of the flame ; if he has exophoria, to the left of it. 

If now the rod is turned at right angles to the position shown in the 
cut and again placed before the right eye, the patient, if he has no 
hyperphoria, will see a horizontal line bisecting the light. If, how- 
ever, he has right hyperphoria the horizontal line will appear below, 
and if he has left hyperphoria it will appear above the light. 

The amount of deviation may be measured by the linear distance 
of the streak from the light (measured on a tangent scale) or by the 
amount of prism that will be required in order to make the streak coin- 
cide with the light. 



202 



THE EYE AND NERVOUS SYSTEM 



The Maddox rod can also be used to indicate whether there is any 
torsion of the eye, — i.e., any tilting of its vertical meridian to the right 
or left. If there is, the streak of light will appear tilted when the rod 
is set directly vertical, and the number of degrees through which the 
rod has to be rotated in order to make the line of light appear vertical, 
will indicate the amount of the torsion. Thus, if the vertical meridian 
of the right eye is rotated 10° towards the temple, we shall have to turn 
the Maddox rod out 10° in order to make the line of light appear 
vertical. 

4. Phorometer. — The phorometer is an instrument for producing 
artificial diplopia by means of prisms, and from the relation of the 
double images deducing whether there is any tendency to deviation or 
not. 

Stevens's phorometer consists essentially of two 6° prisms, geared so 
that they can be rotated simultaneously but in opposite directions. The 
prisms are first set so that their bases both look inward ; the patient 
then looking through the prisms will see two images of the test object, 
that of the right eye being on the right side. If he has no hyperphoria 




the images will be on the same level. If he has right hyperphoria the 
right image will be lower ; if he has left hyperphoria it will be higher. 

In case the images are not on a level the prisms are rotated until 
they are; then the amount of hyperphoria can be read off from the 
index. 

To measure for lateral deviations the prisms are rotated until the 
right-hand one points down, the left up. Then, if there is no esophoria 



THE EXTRA-OCUIAR MUSCLES 203 

or exophoria, the images will appear directly over each other. If, 
however, there is esophoria the upper image will appear on the left; 
if there is exophoria it will appear on the right. In either of the latter 
events the prisms are rotated until the images do stand over each other, 
when the amount of the esophoria or exophoria, as the case may be, can 
be read off from the index. 

5. Bar-reading. — If the patient is directed to read and holds a 
pencil some three inches in front of the page, between it and his eyes, 
he will still, if he has binocular vision, be able to read every word, 




Fig. 8. — Worth's Amblyoscope, modified by Black.— The amblyoscope consists of two tubes, bent 
and joined so as to admit of being converged and diverged through a considerable angle. In the Black 
modification here shown, one of the tubes can also be raised or lowered, an arrangement advantageous 
when the two eyes are not on the same level. My own model is also fitted with a graduated arc 
which shows the amount of the deviation or of the convergence and divergence employed. The fur- 
ther end of each tube carries a picture mounted on a translucent plate of celluloid. A mirror at the 
bent portion of the tube enables the patient looking through the eyepiece to see the picture with the 
corresponding eye. If he has binocular vision he will see two pictures, one with each eye, and by 
winging the tubes in or out he will get these two pictures together and fuse them. 

because the letters that are hidden from the left eye will be seen by the 
right, and vice versa. If he has not binocular vision, the pencil ob- 
viously will cut out part of what he is reading. Hence, this simple test 
enables us to tell whether or not he is using one or both eyes. 

6. Stereoscope and Amblyoscope. — A patient who has binocular 
vision will be able to see two dissimilar images placed in the stereoscope 
and to fuse them into a single image. This, of course, is impossible 
for one who has only monocular vision. Different forms of the stereo- 
scope have been devised, both for determining the presence or absence 



204 



THE EYE AND NERVOUS SYSTEM 



of binocular vision and also for training binocular vision and the fusion 
faculty. 

A similar office is performed much more effectively by the amblyo- 
scope (Eig. 8). 

IV. Tests of Monocular Movements: Field of Fixation. — Roughly, we 
can ascertain how far each eye can move in any given direction by 
making it follow the moving finger or pencil. When we make the test 
in this way we may have to screen the eye not under examination, in 
order to make sure that the one we are testing fixes the moving object 
accurately. Otherwise, the test is performed with both eyes open, and 




Fig. 9.— Stevens's Tropometer. 

we watch the movements 8 both at once, — i.e., the test is really one of 
binocular fixation (V. 1, Page 205). 

More precisely, the range of excursion of each eye separately, and 
consequently the field of fixation of each eye, can be determined by the 
tropometer (Fig. 9), and the perimeter. With the latter the patient 
is placed so that the eye in the primary position is fixing the zero mark 
on the scale ; then a fine object, preferably a pair of dots set very close 
together on a card, is passed along the perimeter arc and the patient di- 
rected to follow it, moving his eyes, but not his head. The moment he 
ceases to follow it, — i.e., the moment that the eye has reached the limit 
of its excursion in that special direction, — the two dots will blur and 
appear to him to run into one. Then the point to which the test-object 



THE EXTKA-OCULAK MUSCLES 205 

has been carried on the perimeter arc shows the amount in degrees that 
the eye has rotated in the given direction. 

By ascertaining the limits of the excursion in different meridians 
the field of fixation may be plotted out on a perimetric chart in the same 
way as is done for the field of vision. 

V. Tests of Binocular Movements. — 1. Tests of Parallel Movements. 
— There are two ways in which the ability of the eyes to perform paral- 
lel movements can be tested. 

Eirst, we may ascertain the field of binocular fixation, — that is, we 
may find how far the two eyes can move in any given direction and keep 
pace with each other in their movements, so that they both fix the same 
object. The usual way of doing this is to make the two eyes follow a 
pencil or similar object in the different directions of the gaze and note 
whether either eye lags or falls behind in making the movement. This 
lagging can usually be detected pretty readily when there is a deficiency 
of either inward or outward rotation. It is well marked, therefore, in 
insufficiency or paresis of the external and internal recti. It is much 
more difficult to tell whether either eye lags behind the other when the 
two are moving up or down. This is especially the case when the eyes 
are directed up and to the right or up and to the left, for then the out- 
ward eye usually appears to be the lower, even when it can be readily 
proved that both are accurately fixing the same object. 

A more accurate means of determining the field of binocular fixa- 
tion is by making the scren test in the various directions of the gaza 
A noticeable deviation behind the screen would show that there was at 
least a tendency to deflection of the eyes. If this deviation was greater 
than in the primary position it would show the deflection was increasing 
as the eyes were carried away from the latter; and if the method of 
binocular uncovering (page 199) should show that there was a squint 
in one of the outlying positions which did not exist in the primary 
position, it would indicate that the deflection had become so great that 
one eye could no longer keep pace with the other and perform fixation 
with it. 

Second, we may determine the field of binocular single vision. In 
doing this we place a red glass before the patient's right eye and stand 
three feet in front of him with a light which we pass to the right, to 
the left, up and to the right, up and to the left, down and to the right, 
and down and to the left. We direct him to follow the light with his 
eyes without moving his head, and if in any direction of the gaze he 
gets double vision, we ask him how the images are related to each other, 
how far they are apart, and whether one or the other is tilted or not. 



206 THE EYE AND NERVOUS SYSTEM 

Normally, the patient should be able to carry the gaze through a 
range of 40° at least in any given direction without seeing double, and 
most people, indeed, will not see double at all, no matter how far the 
test-object is carried. 

Diplopia developing within 30° of the primary position is distinctly 
pathological, and indicates insufficiency of some of the ocular muscles. 

It is sometimes important to determine the field of binocular single 
vision for distance. In this case the patient is made to keep his eyes 
fixed on a small brilliant light fifteen or twenty feet off while he turns 
his head in various directions. A diplopia developed when the head is 
turned to the left, etc., is really a diplopia produced when the eyes are 
turned to the right, etc. (The method of taking and recording is de- 
scribed more fully in Section II. under Diagnosis of Paralysis.) 

In taking the field of binocular single vision we may at the same 
time take the field of binocular fixation, for, as the patient follows the 
light with his eyes, we can, by watching closely, ascertain whether 
either eye seems to lag when the light is carried in any direction. 

2. Tests of Convergence. — These are threefold: (a) By the screen 
and diplopia tests (Maddox rod, phorometer) we measure the amount 
of any deviation that may be present when the eyes are converged on 
a point some ten inches distant. If this deviation is much greater than 
that found for distance by the same tests, it argues some abnormality 
of the converging power, either in the way of excess or defect. Such 
a determination is of great importance and should form a regular part 
of the examination for muscular anomalies. 

(b) We may determine the convergence near-point, or the nearest 
point to which the patient can converge his eyes by the utmost effort. 
This should be not more than two inches from the root of the nose. If 
more than this it argues an insufficiency of convergence. 

Even when, as in divergent squint, the patient has no absolute con- 
vergence near-point, since he cannot produce binocular fixation at any 
distance, far or near, he yet often makes strong converging efforts, 
swinging the eyes nearly but not quite to meet the object of fixation 
as it is brought towards him. The point nearest to the eyes at which he 
gives up this effort, and allows the eyes to diverge widely, is called his 
relative convergence near-point. 

(c) We may determine the prism-convergence (wrongly called the 
adduction). This is measured by the ability to overcome, — i.e., see 
single with — prisms held, base out, before the eyes. 

The amount of prism-convergence that a patient can do at the first 
examination will vary from 10° to 20° or more, but by a little practice 



THE EXTRA-OCULAR MUSCLES 20T 

most people can overcome prisms of 50°, 60°, or more, and this either 
when looking at a distant object or when looking at an object a foot away. 

3. Tests of Divergence. — The ability of the eyes to diverge is meas- 
ured by the prism, base in, that they can overcome. When looking 
at a distant object most eyes can overcome from -±° to 8° of prism, 
base in, this constituting the prism-divergence or so-called abduction 
for distance. 

A prism-divergence of less than 4-° in general indicates weak, and 
of over 8° or 9° an excessive, divergence power. 

How the Tests are made in Practice. — In examining a case for a motor 
anomaly of the eye we proceed, as follows: 

A. We first determine the relations for distance by placing the 
test-object far off, — if possible, twenty feet or more, — from the eyes 
and then measuring the amount of deviation by 

(1) The screen and (2) the parallax simultaneously. At the 
same time we determine by the method described on 
page 21 whether the deviation is a squint or a hetero- 
phoria. 

(3) The Maddox rod, testing both for hyperphoria and for 

lateral deviation. 

(4) The phorometer, testing both for hyperphoria and for lateral 

deviation. 

B. "We measure the deviation for near points (with the test-object 
held at about ten inches) by 

(5) The phorometer; (6) the screen and (7) the parallax 

simultaneously; (2) the Maddox rod. 
In this case, again, we ascertain by the screen test whether 
the deviation is a squint or a heterophoria. 

C. We determine : 

(9) The convergence near-point. 

(10) The prism-divergence, with prisms held, base in, before the 

eyes, the test object in this case being at twenty feet. 

(11) The prism-convergence by means of prisms held, base out, 

before the eyes, the test object again being at twenty 
feet. 

(12) The field of binocular single vision, by using as the test 

object a candle held at three feet from the eye. In 
making this test we note at the same time whether 
the two eyes follow the candle in all directions, or 
whether one eye lags behind the other in performing 
some particular movement, — that is, we determine 
(13) The field of binocular fixation. 



208 THE EYE AND NEKVOTTS SYSTEM 

• Other tests may be employed, but these are sufficient for all practi- 
cal purposes. Indeed, in all ordinary cases we may omit the use 
of the phorometer, and, provided the convergence near-point is nor- 
mal, we may also dispense with the determination of the prism- 
convergence. 

What the Test shows in Normal Cases. — With the test made in the 
way indicated above, the eye-muscles may be regarded as normal if 

(a) Tests 1 and 6 show that there is no squint, and Tests 1 to 8 
show that there is only a minimal amount of heterophoria (less than 
2° of esophoria or exophoria * and less than -J° of hyperphoria) for 
both distance and near. 

(b) The convergence near-point is less than two inches from the root 
of the nose. 

(c) Prism-divergence is not less than 4° and not over 8° (prism = 
2° to 4° of actual deviation). 

(d) Prism-convergence after a few trials can be run up to 15° or 
20° or more.f 

(e) Tests 12 and 13 show that the fields of binocular fixation and 
binocular single vision are normal, — i.e., the patient having a red glass 
before one eye and following a light that is carried in all possible direc- 
tions sees everywhere a single particolored light, and each eye moves 
equally with its fellow without lagging anywhere. 

Anomalies of Eye-Muscles and Eye Movements ; Etiological Clas- 
sification of Deviations.— The various tests just outlined enable us to 
make a diagnosis of the ordinary motor anomalies of the eye commonly 
met with. In considering these anomalies we must distinguish be- 
tween — 

A. Deviations due to anomalies of one or more of the ocular 
muscles; deviations, that is, which are produced by some affection 
of the muscles themselves, of the nerves supplying them, or of the nerve 
nuclei. 

B. Deviations due to derangement of one of the co-ordinating move- 
ments of the eyes. The muscles themselves in this case and their cen- 

* The Maddox rod often shows an excess of esophoria for distance (1-2° more 
than the other tests), and the phorometer sometimes shows 2-3° of exophoria for 
near when the other tests prove that there is orthophoria. 

f There is no absolute or fixed relation between the amount of prism-divergence 
(abduction) and prism-convergence (adduction). The former amounting to from 5° 
to 8°, cannot, as a rule, be altered in any given case by exercise. Prism-convergence, 
on the other hand, may range anywhere from 12° to 30° at the first trial, and, how- 
ever low at the outset, can almost always by practice be brought up to 60° or 70° 
or even more. 



THE EXTKA-OCULAR MUSCLES 209 

tral connections up to and including the nuclei are intact, but the supra- 
nuclear or internuclear nervous mechanism presiding over the functions 
of convergence, divergence, and associated parallel movements is in 
some way out of gear. 

We have, therefore, to consider in succession — 

A. Anomalies of the ocular muscles : 

(1) Paralysis of the ocular muscles. 

(2) Spasm of the ocular muscles. 

B. Anomalies of the co-ordinate movements : 

( 1 ) Anomalies of convergence : 

Convergence-excess. 

Convergence-insufficiency and convergence-paralysis. 

(2) Anomalies of divergence : 

Divergence-excess. 

Divergence-insufficiency and divergence-paralysis. 

(3) Anomalies of parallel movements : 

Conjugate paralysis. 
Conjugate spasm. 
Nystagmus. 

Section II. 
PARALYSIS OF THE INDIVIDUAL MUSCLES. 

Symptoms. — A. Diplopia. — All the subjective symptoms of paralysis 
are due chiefly to the diplopia that the paralysis causes. Since this 
diplopia itself is due to the fact that the eye, weakened by paralysis, 
fails to keep pace with its fellow, and since this diplopia will become 
most apparent as soon as the affected muscle is called into action, it is 
evident that the diplopia, and with it the other symptoms, will increase 
when the eyes are carried into the field of action of the paralyzed 
muscle. Eor example, in paralysis of the right abducens, diplopia will 
increase fast when the eyes are directed to the right ; whereas, when the 
eyes are directed to the left, the diplopia will diminish and ultimately 
disappear, because in that direction of the gaze the affected muscle takes 
no part in moving the eye. 

The characteristic mark of paralysis, therefore, is a diplopia which 
increases progressively and fast as the eyes are carried in some definite 
direction and diminishes when the eyes are carried in the contrary way. 

The kind of diplopia present in the various forms of paralysis will 
be given below under the head of diagnosis. 

The other symptoms beside diplopia which are found in paralysis 
are as follows: 
14 



210 THE EYE AND KEKVOUS SYSTEM 

B. False Projection. — In a fresh case of paralysis of the right ab- 
ducens, the patient when told to close his left eye and quickly put his 
finger on an object situated to his right, will in general shoot too far to 
the right of the object; in other words, the object appears to him to be 
further to the right than it really is. This is due to the fact that in 
order to look at the object with the paretic eye he has to exert an ex- 
cessive amount of nerve energy, such as corresponds in his former 
experience to a marked movement of the eye to the right — that is, he 
has to work hard to move his eye a little and he thinks, therefore, that 
he really, moved it a great deal. 

So, too, in paralysis of a left rotator, the patient will project the 
object too far to the left ; in paralysis of an elevator he will project it 
too high up, and in paralysis of a depressor too low down. 

C. Apparent Movement of Objects pseudokinesis) . — When a patient 
with paralysis of the right abducens looks with his paralyzed eye at an 
object situated to the left, the latter will appear in its proper place ; if, 
then, he suddenly turns his head to the left, so that the object is now 
somewhat to the right, it will no longer appear in its proper place, but 
somewhat to the right of it. In other words, it will seem to make a sud- 
den jump from left to right. And, in general, when the eye is carried 
in a direction corresponding to the field of action of a paralyzed muscle, 
objects at which it is looking will suddenly move, the direction of the 
movement being always that in which the muscle itself would naturally 
turn the eye. That is, in paralysis of a right rotator objects will appear 
to go to the right, in paralysis of a left rotator to the left, in paralysis of 
an elevator they will rise up and in paralysis of a depressor they will go 
down. 

D. Vertigo. — The changing diplopia and the apparent movement of 
objects seen with the paralyzed eye produce vertigo, which is often a 
very marked and distressing symptom of paralysis. 

E. Attitude of Head. — All the symptoms above described increase in 
proportion as the eyes are carried in the special direction in which the 
muscle, if it were not paralyzed, would move the eye. To avoid them, 
the patient instinctively turns his head in this direction so that his eyes 
shall be turned in the opposite way. Thus, in paralysis of a right 
rotator (right externus, left internus), he will turn his head to the right, 
so that even when looking at objects straight in front of him, the eyes 
themselves will be directed to the left. Similarly in paralysis of a left 
rotator, he will turn his head to the left. In paralysis of an elevator, he 
may throw his head up and back, and in paralysis of a depressor, he 
may throw it forward and down. In paralysis of an elevator or de- 



THE EXTKA-OCULAR MUSCLES 211 

pressor, however, the easiest and most usual way of obviating the 
diplopia is for the patient to tilt the head towards one shoulder. This 
depresses the corresponding image,* and thus enables the patient to 
bring the images on a level in case there is vertical diplopia. The head 
at the same time is usually turned somewhat to right or left (Fig. 13). 
This constant maintenance of the head in a constrained position, to 
obviate the diplopia due to a paralysis, has, in some instances, 'produced 
torticollis. (See cases cited by Posey, Section on Ophthalmology, 
Fifty-third Annual Meeting of the American Medical Association.) 




Fig. 13. — Showing torticollis from vertical deviation of 
one eye. Case of Dr. Posey 

F. Other Symptoms. — In paralysis we often meet with other symp- 
toms, such as headache, various forms of anesthesia, paresthesia, etc. 
The headache may occasionally be a reflex symptom, due to the con- 
fusion and strain imposed by the paralysis, but this seems to be very 
rarely the case. Generally speaking, the headache and other symptoms 
are the result of the cause producing the paralysis and not of the 
paralysis itself. 

Diagnosis of Paralysis. — In diagnosticating paralysis we apply 
the various tests already described : 

A. Deviation in Primary Position. — The screen-test and the different 
diplopia tests (parallax, Maddox rod, and phorometer) show the pres- 

* That is,, if he tilts the head to the right shoulder, the right-hand image, 
whether belonging to the right or to the left eye, is carried down. 



212 THE EYE AND NERVOUS SYSTEM 

ence of a deviation even in the primary position. Thus, in paralysis of 
the abducens, the eyes will be unduly converged so that all the tests 
will show a pronounced esophoria; in paralysis of the internus there 
will be exophoria; and in paralysis of the elevators or depressors, 
hyperphoria. 

In applying the screen-test, we will generally notice that the devia- 
tion of the paralyzed eye behind the screen {primary deviation) is less 
than that of the sound eye {secondary deviation). The reason for this 
is as follows: Suppose that the right abducens is paralyzed and that 
this eye, in consequence when screened, deviates inward 1 millimetre ; 
when the screen is transferred to the left eye the right will have to turn 
out 1 millimetre in order to fix. To do this, the eye must employ its 
paralyzed externus and, as this is an inefficient instrument, must exert a 
great deal of nerve power. This same nervous force is necessarily con- 
veyed to the left eye at the same time, impelling it to turn very strongly 
to the right. As the muscles of the left eye are intact, it is able to obey 
this impulse fully, and hence moves to the right, not one but several 
millimetres. 

The difference between the primary and the secondary deviation is 
best measured with prisms. If, for example, a prism of 10°, before the 
right eye will exactly abrogate the screen deviation, but it takes a prism 
of 20° before the left eye to do the same thing, we know that the sec- 
ondary deviation of the left eye is about twice as great as the primary 
deviation of the right. 

B. Limitation of Movement. — The tests for the monocular field of 
fixation made with the tropometer or perimeter show the limitation 
of movement of the affected eye in some particular direction of the gaze. 
This test, however, is often unsatisfactory and is better replaced by the 
following tests for determining the field of binocular fixation and bin- 
ocular single vision. 

C. Lagging of One Eye behind the Other. — The field of binocular fixa- 
tion is determined in the way already described (page 205). In a 
case of paralysis we find that as the two eyes follow a moving object, one 
lags markedly behind the other as soon as the eye is carried in the direc- 
tion of the field of action of the paralyzed muscle. Thus, in paralysis 
of the right abducens the right eye will begin to falter and fall behind 
as soon as the eyes are turned to the right ; in paralysis of an elevator, 
the affected eye will fail to rise with its fellow, and in paralysis of a 
depressor, it will not go down as well. 

By applying the screen-test in the different directions of the gaze, 
it may be possible to show that there is binocular fixation when the eyes 



THE EXTRA-OCULAR MUSCLES 213 

are turned in one way, say the right, and that there is a marked devia- 
tion from binocular fixation when the eyes are turned in the opposite 
way, to the left. This will be evidence of the paresis of a left rotator. 

D. Diplopia. — By far the most satisfactory evidence of paralysis is 
that afforded by the determination of the field of binocular single vision, 
— i.e., by ascertaining the amount and kind of diplopia present in the 
different directions of the gaze. 

The characteristic diplopia found in paralysis of each muscle is as 
follows : 

In paralysis of the right abducens there is homonymous diplopia, 
increasing fast to the right. In paralysis of the left abducens there 
is homonymous diplopia which increases fast to the left. In both cases 
the diplopia will be slightly less when the eyes are directed up and 
slightly greater when the eyes are directed down. 

In paralysis of the right internal rectus there will be crossed di- 
plopia increasing fast to the left. In paralysis of the left intemus there 
will be crossed diplopia increasing fast to the right. In both cases the 
crossed diplopia will increase somewhat when the eyes are turned up 
and diminish somewhat when they are turned down. 

In paralysis of the right superior rectus there will be vertical di- 
plopia, with the image of the right eye higher (left diplopia). This will 
increase as the eyes are carried up, and particularly as they are carried 
up and to the right. In general, but not invariably, there will be also a 
moderate crossed diplopia, which will increase in looking to the left. 
The image of the right eye will be tilted to the left. 

In paralysis of the left superior rectus there will be a vertical di- 
plopia (right diplopia), increasing up and to the left, and a crossed 
diplopia, increasing up and to the right. The image of the left eye 
will be tilted to the right. 

In paralysis of the right inferior rectus there will be a vertical 
(right) diplopia, increasing as the eyes are carried down and to the 
right, and usually, but not always, a crossed diplopia, which will increase 
in looking down and to the left. The image of the right eye will be 
tilted to the right. 

In paralysis of the left inferior rectus there will be a vertical (left) 
diplopia, increasing down and to the left, while the crossed diplopia, if 
present, will increase in looking down and to the right. The image of 
the left eye will be tilted to the left. 

In paralysis of the right superior oblique there will be a vertical 
(right) diplopia, which will increase as the eyes are directed down and to 
the left, and, usually, also an homonymous diplopia, which will increase 



214 THE EYE AND KEKVOUS SYSTEM 

down and to the right. The image of the right eye will be tilted to 
the left. 

In paralysis of the left superior oblique there will be a left diplopia, 
increasing down and to the right and an homonymous diplopia, in- 
creasing down and to the left. The image of the left eye will be tilted 
to the right. 

In paralysis of the right inferior oblique there will be a left di- 
plopia, which will increase as the eyes are carried up and to the left, 
and sometimes, but not always, an homonymous diplopia, which will 
increase as the eyes are carried up and to the right. The image of the 
right eye will be tilted to the right. 

In paralysis of the left inferior oblique there will be a right di- 
plopia, increasing up and to the right, and an homonymous diplopia, 
increasing up and to the left. The image of the left eye will be tilted 
to the left. 

We meet with various combined paralyses producing more or less 
complicated forms of diplopia. Chief among these is the oculo-motor 
paralysis, or paralysis of all the muscles supplied by the third nerve. 
In this case the eye can be moved outward by the abducens, but it can- 
not be moved in any other way, for, although the superior oblique is 
still acting, it cannot move the eye downward since it is not efficient as 
a depressor when the eye is in a position of abduction, as it is in paral- 
ysis of this sort. The most that the superior oblique can do under these 
conditions is to give the eye a slight inward rotation upon its antero- 
posterior axis when the patient attempts to turn the eye down. 

In a right oculo-motor paralysis there will be a crossed diplopia, due 
to the relative divergence of the paralyzed eye, and usually also a slight 
left diplopia, because the eye sags somewhat below the level of the 
other. This left diplopia will increase fast as the eyes are carried up. 
On the other hand, when the eyes are carried down, the left diplopia 
will be transformed into a vertical diplopia of the opposite kind (right), 
which will increase fast as the eyes are depressed. 

The diagnosis of a paralysis from the behavior of the double images 
will be facilitated by the following table : 

TABLE SHOWING FROM DIPLOPIA WHICH MUSCLE IS PAKALYZED. 

DH=homonymous diplopia. 
DX=crossed diplopia. 

DR=right diplopia (vertical diplopia with image of right eye below). 
DL=left diplopia (vertical diplopia with image of left eye below). 
Er, El, Eu, Ed, Eu and r, etc.=respectively " when both eyes are directed to the 
"right" "to the left, 11 "up, 11 "down, 11 " up and to the right, 11 etc. 
»=increasing fast and progressively. 



THE EXTRA-OCULAR MUSCLES 215 

A. The diplopia is lateral (DX or DH) » in Er or El. (= paralysis of a lateral 

rotator.) 

Diplopia » in Er. (=paralysis of Diplopia » in El. (=paralyeis of a 

a RIGHT ROTATOR. ) LEFT ROTATOR. ) 

DH » in Er. E. externus. DH » in El. L. externus. 

DX » in Er. L. internus. DX » in El. E. internus. 

B. The diplopia is vertical (DR or DL) » in Eu. (=paralysis of an elevator.) 

Diplopia » in Er (Eu and r). Diplopia » in El (Eu and 1). (=pa- 

(=paralysis of a right-hand ele- ralysis of a left-hand elevator). 

vator. ) 

DL » in Eu and r. E. superior DR » in Eu and 1. L. superior 

rectus. rectus. 

DR » in Eu and r. L. inferior DL » in Eu and 1. JR. inferior ob- 

oblique. lique. 

C The diplopia is vertical (DR or DL) » in Ed. (=paralysis of a depressor.) 

Diplopia » in Er (Ed and r). Diplopia » in El (Ed and 1). (=pa- 

(=paralysis of a right-hand de- ralysis of a left-hand depressor.) 

pressor. ) 

DR » in Ed and r. E. inferior DL » in Ed and 1. L. inferior rectus. 

rectus. 

DL » in Ed and r. L. superior DR » in Ed and 1. E. superior ob- 

oblique. lique. 

A good rule to enable us to determine which is the paralyzed eye is 
the following: 

When, in moving the eyes in a given direction, the double images 
begin to separate, that image which moves the faster and the further 
belongs to the paralyzed eye. 

And a rule to determine the kind of double vision produced by a 
given paralysis is, — the image belonging to the paralyzed eye is dis- 
placed in just that direction in which the affected muscle, if intact, 
would naturally move the eye. Thus, in paralysis of the right superior 
rectus, which moves the right eye up and to the left and tilts the verti- 
cal meridian to the left, the image of the right eye is up (higher), de- 
flected to the left (crossed diplopia), and tilted to the left. 

The double images may be conveniently plotted on a diagram, as 
shown in Eig. 14. 

Fixation with the Paralyzed Eye. — A marked change in the ap- 
pearances presented, although not in the actual conditions present, is 
produced by fixation with the paralyzed eye. The paretic eye, in this 
case, is forced into position, and the sound eye deviates. Xow the force 
applied to the sound eye to make it deviate is equal to the force applied 
to the paretic eye to make it get into position. This latter force, how- 
ever, must be very considerable, and the more so the greater the degree 



216 



THE EYE AND NERVOUS SYSTEM 



of paresis. Hence, as we have seen (p. 212), the deviation of the sound 
eye when the paretic eye fixes (secondary deviation) is much greater 
than the deviation of the paretic eye when the sound eye fixes (primary 



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Fig. 14.— Diagram for plotting Double Images in Paralysis.— The diagram is made on the 
assumption that the field of binocular single vision is taken at a distance of thirty inches and pro- 
jected on a tangent plane at that distance (represented by the large square). The fine cross lines 
denote intervals of 2 inches ; the heavier cross lines, intervals of one foot. The concentric circles 
denote intervals of 5° plotted on the tangent plane. The diagram is used as follows : The light is 
held thirty inches from the patient and carried into the six cardinal positions "Eyes right" (Er), 
"Eyes up and right" (Eu and r), etc., and the amount of diplopia, vertical and lateral, in inches is 
laid off on the diagram. In the case shown (one of paralysis of the left superior rectus), in which the 
image of the right eye is denoted by a circle and that of the left by a cross, the vertical distance be- 
tween the two images when the eyes are directed two feet up and two feet to the left is six inches 
and the lateral distance is four inches. The diagram shows that this means that when, the eyes are 
elevated 38° and also carried 38° to the left, the vertical diplopia is over 5° and the lateral diplopia 
about 3° of arc. When the eyes are directed the same distance up and to the right there is simple 
crossed diplopia of 5°, and if directed two feet straight to the left a simple vertical diplopia of 2.5°. 
Below the oblique line AB (that is in the positions Er, Ed and r, and Ed and 1) there is single vision. 
The diagram can be used for any other distance than thirty inches, provided the proper allowance is 
made. Thus if the light were held five feet from the patient, the fine cross lines would represent 
intervals of four inches, and the heavy cross lines intervals of two feet. 



deviation). Accordingly, the effect of fixation with the paralyzed eye 
is to increase the deviation sometimes to double or treble of its original 
amount. The result is that the double images are put much further 



THE EXTKA-OCULAK MUSCLES 217 

apart, and hence interfere less with each other, cause less confusion, and 
are more readily ignored. This, in fact, as Graefe says, is evidently the 
reason why the patient adopts this manoeuvre at all. 

This secondary deviation of the sound eye, when the paretic eye 
fixes, always appears under the guise of a spasm of the associate (page 
17) of the paralyzed muscle. Thus, if the right superior rectus is 
paralyzed, and yet this eye is used for fixation, the appearances pro- 
duced will be those of a spasm of the left inferior oblique. For, in this 
case the right eye tends all the time to drop and can be kept in place 
only by an excessive nervous effort. This effort will be very great when 
the eyes are directed to the right, because in this position its paretic 
superior rectus is the only muscle that lifts the right eye. On the other 
hand, when the eyes are directed to the left, the right superior rectus 
acts very little as an elevator, and, as the muscle (the inferior oblique) 
which here does the main part of the work is normal, but little extra 
effort will be required to keep the eye in place. Now the extra effort 
that is put forth to keep the right eye in place is exerted equally upon 
the left eye. Hence, when looking to the right the left eye will rise 
unduly (shoot obliquely up), while in looking to the left, it will rise 
little or not at all above the proper level. This is just the effect 
that would be produced by a spasm of the right-hand elevator 
(inferior oblique) of the left eye. Similarly, as shown in the table 
on page 246, a paralysis of the right superior oblique will, if the right 
eye is fixing, give the appearance of a spasm of the left inferior 
rectus, etc. 

Fixation with the paralyzed eye is not infrequent. It is especially 
apt to occur when the paretic eye has the better vision, although it may 
also occur when this is the poorer eye of the two. It seems to be com- 
paratively frequent in paralysis of the superior rectus, and not very 
frequent in abducens paralysis. 

Sometimes the patient fixes alternately with the paretic and the 
sound eye. Thus, in one case that I saw, a boy with a paresis of the 
left superior rectus, either fixed with the right eye so as to have a left 
hypotropia, or with the left eye so as to have a right hypertropia (alter- 
nating vertical squint). In the same way an alternating convergent 
squint may be produced by alternate fixation in a case of paralysis of 
one abducens. 

Varieties of Paralysis. — Isolated and Combined Paralyses. — Paralysis 
may affect one or several ocular muscles. 

Isolated paralysis of the abducens is very common and such paraly- 
sis is occasionally bilateral. 



218 THE EYE AKD NERVOUS SYSTEM 

Oculomotor paralysis, complete or partial, is the next most frequent 
form, and all sorts of combinations occur. In complete oculomotor 
paralysis, not only are the motions of the eye affected, as stated (page 
39), but there are also ptosis (or drooping of the lid from paral- 
ysis of the levator), paretic mydriasis, and paralysis of accommodation. 
Frequently the accommodation and the iris alone are paralyzed (oph- 
thalmoplegia interior or interna) (see page 312) ; in other cases just 
these muscles are exempt. In other cases still, ptosis alone is present 
and is often bilateral. In ptosis not only is there complete or partial 
drooping of the lid, but there is also considerable wrinkling of the 
brow, because the patient endeavors by compensatory contraction of the 
frontalis to make up for the deficient action of the levator. 

The next most frequent form of paralysis in the author's experience 
(differing therein from that of other observers) is isolated paralysis of 
the superior rectus. It also is sometimes bilateral. 

Next in order comes paralysis of the superior oblique (trochlear 
paralysis). This is fairly common, but it is very rarely indeed bi- 
lateral, only two or three such cases being recorded, one of which 
occurred in the author's own experience. 

Isolated paralysis of the inferior rectus is fairly frequent, occurring 
in the author's experience more often than that of the internal rectus. 

Isolated paralysis of the inferior oblique is very uncommon. Some 
thirty cases have been recorded, but less than half of these are authen- 
tic. Yet it is probably more frequent than has been supposed, since the 
author has seen no less than four cases, all incomplete. One of these 
was congenital. In a single instance, reported by Bielschowsky, the 
paralysis was bilateral. 

In some cases there is total paralysis of all the muscles moving the 
eyeball and of the interior muscles as well (total ophthalmoplegia). 
The eyeball is then perfectly motionless, the pupil widely dilated and 
the accommodation entirely absent. More or less complete paralysis of 
all the exterior muscles with exemption of the sphincter and ciliary 
muscles, is called ophthalmoplegia exterior or externa. 

Etiology. — Etiologically, we have to distinguish three varieties of 
paralysis, — structural, insertional, and innervational. 

Structural paralysis is due to some abnormality of the muscle itself. 
Such abnormality may be the result of congenital defect, solution of con- 
tinuity, inflammation of the muscle or its surroundings, atrophy, etc. 
(For fuller details see section on Orbital Paralysis.) 

Insertional paralysis is the form in which the muscle and its supply- 
ing nerve are normal, but the muscle is improperly inserted, either as 



THE EXTKA-OCULAK MUSCLES 219 

the result of congenital misplacement, accident, or operation. (See 
for fuller details Orbital Paralysis.) 

Innervational paralysis is the form in which the muscle and tendon 
are normal, but there is some abnormality of the nerve or its central 
connections. Such abnormality may consist in (a) congenital defi- 
ciency; (b) solution of continuity; (c) ana?mia or hyperemia, due 
either to general disturbance of the cerebral circulation, or to pressure 
upon the vessels supplying the nerve or its nucleus; (d) atrophy of the 
nerve or its nucleus, due to pressure of exudates, inflammatory swell- 
ings, callus, hemorrhages, aneurismal, atheromatous or thrombotic 
vessels, or neoplasms; (e) softening or degeneration of the nerve or its 
nucleus. 

With regard to site we divide paralysis into extracranial and intra- 
cranial. 

Extracranial (Orbital or Peripheral) Paralysis. — In a certain number 
of cases orbital paralysis is congenital. (For varieties see below under 
Exciting Cause, Congenital Conditions.) 

Orbital paralysis (or insufficiency) may be due to developmental 
changes in the muscles or nerves. One variety of this is the relative 
insufficiency of the interni and preponderance of the externi, caused 
by the gradual separation of the orbits, which takes place in the 
development of the skull in childhood. This separation, as Weiss 
showed, by giving the externi a larger and the interni a smaller arc 
of contact with the eyeball, tends to produce a relative divergence of 
the eyes. 

Some orbital paralyses are due to traumatism, either from the 
direct effect of the injury upon the muscle or tendon, or the remote 
effects produced by the pressure of effused blood and callus in the case 
of fractures, or to cicatricial contraction in the tissues of the orbit. 
(See for further particulars Exciting Causes, Traumatism.) 

Orbital paralysis may, very rarely, be due to disease of the muscles 
themselves, as to myositis, simple (Gleason) or syphilitic (Hochheim) ; 
to hyaline degeneration (Nuel) ; to degenerative changes occurring in 
progressive muscular dystrophy (Groenouw; UhthofT) and in acute 
ascending paralysis (Goebel) ; and to metastatic tumors (six cases cited 
by Groenouw and Bernheimer). 

In other cases it is produced by disease of the nerves, especially a 
primary tabic degeneration (Dejerine, Marina) ; very rarely by second- 
ary new growths occurring in the nerves (Bernheimer). 

A more usual cause of orbital paralysis is the presence of tumors 
(especially gummata, cavernous growths, exostoses, and sarcomata) or 



220 THE EYE AND NEKVOUS SYSTEM 

inflammatory processes (orbital cellulitis, tenonitis, periostitis, muco- 
cele) of the contents, walls, and accessory cavities of the orbit. These 
processes may act by causing secondary changes (inflammation, de- 
generation, or atrophy) in the muscles and nerves, in which case the 
picture presented will be that of a true paralysis of some muscle or 
group of muscles. They often, however, act in a purely mechanical 
way by pressing the eyeball forward or by enveloping it and its muscles 
in rigid masses preventing movement. In this case the deviation of the 
eye, the restriction of movement, and the diplopia produced may differ 
altogether from what we find in any of the types of paralysis we have 
described. 

A similar mechanical restriction of eye movements with more or 
less aberrant diplopia may be caused by a pterygium or pseudo- 
pterygium. 

Intracranial Paralysis comprises basal, nuclear, and cortical paralysis. 

Basal (and Foraminal) Paralysis. — By basal paralysis we understand 
a paralysis due to lesion of the nerve-trunk after its emergence from 
the brain and before its exit from the skull ; by a foraminal paralysis, 
one due to lesion of the nerve as it passes through the sphenoidal 
fissure. The nerves in traversing this fissure pass through veritable 
foramina, whose walls are made up of rigid fascia. This arrangement, 
while protecting them from injury, renders them more liable to com- 
pression and strangulation. 

Basal and foraminal paralyses are due to — 

(1) Arrested or imperfect development of the nerve-trunks (con- 
genital paralysis). Very rare. 

(2) Solution of continuity of the nerve from blows on the head 
causing fractures of the base, or from shot or stab wounds of the mouth 
or orbit. 

(3) Hemorrhage into the nerves from traumatism, or from the 
spontaneous rupture of aneurismal or sclerotic vessels. 

(4) Inflammation or degeneration of the nerves from syphilis or 
other causes. 

(5) Tumors of the nerves. 

(6) Pressure on the nerves from (a) hemorrhage; (b) inflamma- 
tory exudate (meningitis, periostitis) including callus; (c) hard or 
distended vessels; (d) tumors (especially gummata) ; and (e) general 
distention of the brain. 

Nuclear and Fascicular Paralyses. — By nuclear paralysis we under- 
stand a paralysis due to lesion of the nerve-nucleus itself; by fascicular 
paralysis, one due to lesion of the root-fibres which emanate from the 



THE EXTRA-OCULAR MUSCLES 221 

nucleus and which, before leaving the brain, coalesce to form the nerve- 
trunk. 

Nuclear and fascicular paralyses are due to — 

(1) Aplasia of the nucleus (congenital paralysis). Very rare. (It 
must be noted that, as Wilbrand and Saenger point out, some cases of 
congenital nuclear paralysis are due not to aplasia but to degeneration 
and atrophy of the nucleus from fetal or infantile disease. The dis- 
tinction between the two would be that in aplasia there would not be, 
and in acquired atrophy there would be, a secondary denegeration of 
the nerve-trunk and muscles). (Compare Chapter XIII, Sect. II.) 

(2) Atrophy and degeneration of the nucleus and root-fibres from 
syphilis, tabes, multiple sclerosis, and rarely from other cerebro-spinal 
diseases; also from poisons and the toxins of diseases like diphtheria, 
influenza, etc. (Compare Chapters XII and XV). 

(3) Anosmia, hypercemia, and hemorrhage, due usually to vascular 
changes (arteriosclerosis, endarteritis, miliary aneurisms), which are 
themselves generally the results of syphilis or the action of poisons 
(lead, diphtheria, influenza, etc.). A peculiar form of hemorrhagic 
disease of the nuclei and root-fibres is polioencephalitis superior (see 
below under Exciting Causes). Xuclear hemorrhages may also be due 
to traumatism. 

(4) Tumors, especially gumma, more rarely glioma, sarcoma, and 
tuberculoma (Compare Chapter IX). 

It must be noted that owing to the connection between the abducens 
nucleus of one side and the opposite oculo-motor nucleus, a lesion 
(tumor, etc.) of the pons involving, say, the right abducens nucleus, does 
not cause simple paralysis of the right externus, but a conjugate paraly- 
sis (paralysis of dextroversion in both eyes). To produce simple pa- 
ralysis of the abducens, the lesion must be so situated as to cut off the 
emergent root-fibres of the nerve ; in other words, it must be a fascicular 
paralysis. 

Supranuclear and Cortical Paralyses. — Lesions affecting the connections 
of the nuclei with the cortex and the cortex itself do not in general 
cause paralysis of the individual ocular muscles, but paralysis of the 
co-ordinated movements of the eye, — i.e., of convergence, divergence, 
or the parallel movements. An exception seems to be ptosis, which in 
some twenty-five reported cases (Willbrand and Saenger) has been 
caused apparently by a cortical lesion and particularly one situated in 
the supramarginal and angular gyri (usually on the side opposite to 
the ptosis). Doubt, however, has been thrown on these cases, first, 
because often marked lesion of these parts may exist without causing 



222 THE EYE AND XEBVOUS SYSTEM 

ptosis; and, second, because even when ptosis is present we cannot 
always be sure that is not due to a remote effect (nuclear or basal 
lesion) produced by the cortical disease (Compare Chapter IX). 

Exciting Causes of Paralysis. — The causes producing the various 
lesions enumerated above as present in the different forms of paralysis, 
orbital, basal, and nuclear, are as follows: 

1, Congenital Conditions. — Congenital paralysis in most cases prob- 
ably is due to non-development or faulty development of the ocular 
muscles themselves (orbital paralysis). The congenital malformations 
of the muscles causing paralysis are as follows : 

(a) Absence of the muscle, — e.g., to absence of the internus 
(Krause, Lawford), the externus (Krause, Bahr), the superior rectus 
(Seiler, Steinheim), both inferior recti (Stieren, A. E. Davis), and one 
or both obliques (Hartes, Seiler). 

(b) Eeplacement of muscle by a more or less inelastic cord of con- 
nective tissue. This has been observed in the case of the internus 
(Uhthoff, Guende), and the externus (Baumgarten, Inouye, Ailing, 
Axenfeld and Schurenberg, Evans, Bernheimer). 

(c) Underdevelopment of the muscle. This has been observed by 
Lawford, Bach, and others. A moderate degree of underdevelopment is 
considered by Schneller to be a frequent cause of squint {structural 
squint). This view he bases on the findings in numerous squint opera- 
tions. It is questionable, however, whether the underdevelopment that 
he found was not rather the result than the cause of the squint. 

(d) Abnormal course and insertion of the muscle. Among the 
abnormal conditions found are: (1) Insertion of the muscle too far 
back, causing it to act as a retractor rather than a rotator of the eye 
(Heuck) ; (2) bifurcation of the tendon (Dieffenbach, Bahr *), and 
(3) the presence of adhesions or bands connecting the muscles with 
each other or with the orbital walls (Morgagni, Olbers and Wrisberg, 
Fitzgerald). 

Much more rarely congenital paralysis is due to aplasia of the nerves 
(either in the orbit or the cranial cavity) or of their nuclei. 

Some cases of congenital paralysis are traumatic, being produced 
either by direct pressure or by hemorrhage during delivery with the 
forceps. Such paralysis may affect the external rectus with or with- 
out involvement of the facial nerve, or the oculo-motor nerve, or the 
superior rectus with the levator (Groenouw, Nettleship). 

Other rare cases of congenital paralysis are apparently due to fetal 

* In Bahr's case one of the slips was inserted too far back, causing the muscle- 
to act partly as retractor. 



THE EXTKA-OCULAR MUSCLES 223 

inflammation or degeneration of the nerve nuclei (Wilbrand and 
Saenger). 

Congenital paralyses may occur under all kinds of forms (Kunn, 
Zweig). The following comprise some of the more frequent types. 

A. Paralysis of the Superior* Rectus. — This is either unilateral or 
bilateral ; partial or more often complete ; and may or may not be 
associated with ptosis. This condition is quite frequent. It is prob- 
ably due in most cases to underdevelopment or actual absence of the 
muscle. 

B. Ptosis. — This is nearly always bilateral. It is usually incom- 
plete, the lid simply drooping enough to give the patient a sleepy look. 
Sometimes, however, as in Lawford's cases, the eye is completely closed. 
Congenital ptosis is often combined with other defects, particularly with 
paralysis of the superior rectus. 

It may be nuclear in origin, but is probably in most cases due to 
structural defects in the muscle itself (absence or conversion into con- 
nective tissue, underdevelopment, restriction by adhesions). 

C. Paralysis of the Externus with Paresis of the Internus and 
Retraction Movements. — This condition usually presents itself under the 
guise of a well-marked syndrome characterized by some or all of the 
following features : 

(1) The affected eye can move out little, if at all, beyond the 
median line. 

(2) Its movement inward is more or less restricted. Moreover, 
even when the amount of movement inward is nearly normal, the 
movement, itself is performed with difficulty, the eye always lagging 
behind its fellow, as shown by the presence of a crossed diplopia, in- 
creasing as the eye is turned in. 

(3) When the affected eye is turned in, it retracts into the orbit 
and often also shoots obliquely up or down, and its palpebral fissure 
contracts, often considerably. 

(4) The power of convergence is often considerably impaired. 

(5) The field of single vision is very narrow indeed. The patient 
when he looks to the side of the affected eye has homonymous diplopia, 
which passes almost at once into a crossed diplopia when he begins 
looking in the opposite direction. 

These cases are not uncommon, some fifty-five having been described 
(Duane). Usually the left eye, rarely both eyes, are affected. 

The underlying cause of this condition is a congenital absence of the 
external rectus or its transformation into a connective tissue cord. In 
some cases this cord is elastic and the eve can be rotated inward by 



224 THE EYE AND NERVOUS SYSTEM 

passive traction (with forceps) ; in other cases where the cord is in- 
elastic this is not possible. 

The retraction of the eyeball when addncted is due in most cases 
undoubtedly to the fact that the externus is thus inelastic, so that the 
eyeball, being hugged between an inextensible cord on the one side and 
the contracting internus on the other, must necessarily recede (Turk, 
Wolff). Other cases, however, are due to the fact that the internus is 
inserted too far back (Heuck, Evans), or has an additional slip that is 
so inserted (Bahr, Axenfeld and Schiirenberg). 

D. Somewhat analogous to the condition just described is that 
known as strabismus fixus. In this the eye is turned strongly in and can- 
not be turned outward even by traction with forceps (Berry). Opera- 
tion has no effect, the eye being evidently fixed in its deviating con- 
dition by strong adventitious bands. This condition tends to grow 
worse in after life and is not always congenital (Fisher, Donaldson, 
De Vincentiis). 

There are certain peculiarities distinguishing congenital from ac- 
quired paralyses. These are according to Kunn (cited by Wilbrand 
and Saenger, and Groenouw) : 

(1) Absence of secondary contracture (page 243). This is a usual 
but not an invariable sign, for secondary contracture may be absent in 
acquired and present in congenital paralysis (A. Graefe). Correspond- 
ing to this absence of contracture is the fact that there may be no devia- 
tion in the primary position, or there may be a deviation contrary 
to what we should expect. But such an anomalous placing of the 
eyes in congenital paralysis seems, often at least, to be due to com- 
plications which would produce a like result if the paralysis were 
acquired. 

(2) Retention of convergence in spite of the loss of ability to per- 
form lateral movements. This is by no means a sure differential sign. 

(3) Absence of secondary deviation in the sound eye. But many 
cases of congenital paralysis present a well-marked secondary deviation 
(Duane). 

(4) Absence of spontaneous diplopia. This again is by 'no means 
the case in all congenital paralyses. 

(5) Sudden spasmodic action of the apparently paralyzed muscle. 

(6) Absence of any involvement of the iris and ciliary body. This 
speaks strongly for an orbital origin of the paralysis (structural paraly- 
sis). If the paralysis were nuclear we should expect the interior 
muscles to be at least occasionally affected. 

A point laid down by Werthheim (cited by Zweig) is th^t if 






THE EXTRA-OCULAR MUSCLES 225 

ptosis is present, the levator will show a reaction of degeneration in 
acquired but not in congenital paralysis. 

Other alleged differential points, such as the frequent presence of 
nystagmus or of impaired vision in cases of congenital paralysis, are 
of no real diagnostic value. 

2. Heredity. — The effect of heredity is seen in some forms of con- 
genital paralysis, particularly ptosis, which show a distinct tendency 
to run in families. The same thing is occasionally seen in acquired 
paralysis, — e.g., in acquired ptosis (Delord) and in a case reported by 
Beaumont, in which ophthalmoplegia exterior occurred regularly in sev- 
eral members in each of four generations of the same family. 

Heredity also plays an important part in inducing the conditions 
(arteriosclerosis, syphilis, etc.) which frequently lead to paralysis (see 
infra). 

3. Traumatism. — This is a fairly frequent cause of paralysis. 
Paralysis of the external rectus is most frequent and may be bilateral. 
It is sometimes evidently orbital in origin, being due to injury of the 
muscle itself or the orbital section of the abducens nerve ; at other 
times, especially when bilateral, is basal, being due particularly to 
fractures through the petrous bone. Traumatic paralysis, complete or 
partial, of the third nerve is also often basal, being due to fracture of 
the base of the skull or penetrating wounds through the orbit or mouth. 
Isolated paralysis of the superior or inferior rectus or of the inferior 
oblique is also frequently (although not, as has been asserted in the 
case of the latter muscles, invariably) the result of traumatism. The 
injury here usually affects the muscle itself or the nerve-terminals in 
the orbit (orbital paralysis). The same is true of the not infrequent 
traumatic ptosis. 

Traumatic paralysis of the superior oblique is rather uncommon. 
It may be basal or orbital — one variety of the latter being the paralysis 
sometimes observed after operations upon the frontal sinus and caused 
by injury to the trochlea. 

Traumatic paralyses are due either to direct injury of the muscle or 
nerve, in which case they are found immediately after the injury, or to 
compression by displaced bone, effused blood, callus, traumatic cerebral 
abscess, or a traumatic aneurism (especially of the internal carotid), 
in which case they may not develop until some time later. In some 
cases, but probably not often, traumatic paralysis is nuclear, being due, 
according to Bernheimer, to distention of the ventricles consequent 
upon the injury and leading to hemorrhage into the nuclei adjoining. 

As above noted (page 222), traumatic paralysis may be congenital. 
15 



226 THE EYE AND NEKVOUS SYSTEM 

Some cases of traumatic paralysis, especially when due to injury of 
the face, are associated with enophthalmos. This, as Purtscher says, 
may be caused by (a) retraction due to scar-tissue; (b) loosening 
of the check-ligaments or the insertions of the obliques; (c) displace- 
ment of the contents of the orbit; (d) paresis of the sympathetic (in 
this case there will be unilateral miosis) ; (e) trophic changes from 
injury to the peripheral nerves. 

One variety of traumatic paralysis that we meet with pretty often 
is that produced by a tenotomy. We must, in fact, remember that 
whenever we produce an effective tenotomy we really produce a paresis 
of the muscle. The action of the check-ligaments and of the adminicula 
of the tendons prevents this paresis from becoming complete, and when 
the tendon re-attaches itself it is able to work on the eyeball as before, 
although not as effectually. Nevertheless, even after re-attachment, if 
the operation has had any effect at all, we can usually demonstrate 
restriction of movement such as would be caused by paresis of the 
muscle, and in many cases also a characteristic diplopia. This is espe- 
cially seen after tenotomies of the superior and inferior recti. In these 
for years after the original operation we can in many cases demonstrate 
diplopia in one quadrant of the field of fixation. 

The fact that a tenotomy has caused a paralysis may often be in- 
ferred from (a) the presence of a conjunctival scar or of sub-con junc- 
tival adhesions. If not demonstrable by inspection these can be readily 
made out by exploration through a trifling and harmless incision in the 
conjunctiva made under cocaine, (b) There may be slight exophthal- 
mus, particularly when the eye is at rest, (c) In paralysis of the 
superior or inferior rectus, due to a tenotomy, the palpebral fissure is 
wider than normal, since with the retraction of the tendon, its palpebral 
band also retracts and pulls the lid back. In paralysis of these muscles 
due to any other cause, the palpebral band is drawn upon less than 
usual, because the muscle to which it is attached is flaccid, and hence 
the palpebral fissure is narrower than normal. 

4. Vascular Changes in the Brain. — Paralysis, both basal and nuclear, 
may be caused by — 

(a) Hyperemia and ancemia of the brain. These produce often 
very transient paralyses (Bernheimer). 

(b) Embolism. 

(c) Sclerosis of the vessels due to senile changes, syphilis, lead- 
poisoning, etc., causes paralysis by pressure or more often by hemor- 
rhage due to their rupture. 

(d) Aneurisms either (1) of the internal carotid or posterior cere- 



THE EXTRA-OCULAR MUSCLES 227 

bral, or (2) miliary aneurisms of the small vessels. The former act 
by pressure. Aneurism of the internal carotid causes usually gradual 
paralysis of the third nerve, combined with paralysis of the fifth ; less 
often paralysis of the fourth and sixth nerves. Miliary aneurisms, 
which are frequently due to syphilis or to the effect of alcohol, tobacco, 
or other deteriorating influences, give rise to hemorrhages producing 
basal or nuclear paralyses. 

5. General Distention of the Brain, or Increase of Intracranial Pressure. 
— Abscesses or tumors in the interior of the brain, or other processes 
(hydrocephalus) which are not so situated as to cause direct pressure 
on the nerves, yet may induce paralysis by indirect pressure, especially 
if at the same time there are other conditions (arteriosclerosis, etc.) 
which favor the development of paralysis. In such a case lumbar 
puncture may relieve the condition (Brieger cited by Eversbusch). 

6. Inflammation of the Brain and Membranes. — (a) Encephalitis of 
adults and of children (inflammatory cerebral softening, cerebral in- 
fantile paralysis) is an occasional cause of paralysis. 

(b) Abscess of the brain, either traumatic or otitic; especially the 
otitic abscess of the temporal lobe, which may cause paralysis of the 
third (rarely of the sixth) nerve. It is often associated with other 
evidences of pressure, especially optic neuritis and a crossed hemiplegia. 

(c) Polioencephalitis superior, a condition of hemorrhagic infiltra- 
tion of the gray matter about the ventricles, producing destruction first 
of the association tracts and then of the nuclei, and ending in complete 
or nearly complete ophthalmoplegia. It occurs particularly in drinkers, 
but may also be caused by influenza. It is usually but not invariably 
fatal. 

(d) Meningitis, syphilitic, tuberculous or cerebrospinal. The 
nerves at the base may be affected either because they are compressed 
by the exudate or because they become secondarily inflamed. Cerebro- 
spinal meningitis is especially apt to cause abducens paralysis and 
ptosis. 

7 # Other Diseases of the Brain producing Ocular Paralyses. — (a) 
Acute bulbar paralysis causing often paralysis of the abducens, and 
not infrequently of the oculo-motor nerve also. 

(b) Myasthenia gravis. In this evidences of paresis of the ocular 
muscles (especially ptosis) are very frequent and are often the initial 
symptoms. 

(c) Progressive amyotrophic bulbar paralysis and pseudobulbar 
paralysis very rarely cause ocular paralyses. 

8. Tumors of the brain or skull, especially glioma, tuberculoma, and 



228 THE EYE AND KERVOUS SYSTEM 

gumma, of the brain; gumma, exostosis and sarcoma in the cranial 
cavity and orbit; and especially tumors of the hypophysis (acromeg- 
aly.) In some cases, according to Bernheimer, these tumors act not 
only by causing pressure, but also by virtue of the toxins which they 
discharge into the circulation. In cases of basal paralyses due to 
tumors, the third nerve is particularly apt to be affected. Involvement 
of other cranial nerves (second, fifth, seventh, and twelfth) is common, 
and optic neuritis is very frequently although not invariably present. 

Tumors of the pons readily give rise to abducens paralysis, often 
combined with paralysis of the facial and with crossed hemiplegia, and 
not infrequently with involvement of the fifth, eighth, and twelfth 
nerves. 

Rarely, as we have already seen (page 219), paralysis may be due to 
metastases of tumors taking place in the nerves or the muscles them- 
selves. 

9. Spinal Diseases. — Chief among these are — 

(a) Tabes. — This is a very frequent cause of paralysis of the eye 
muscles both interior (see Chapter XII) and exterior. According to 
Uhthoff, one-fifth of all ocular paralyses are tabic and rather more than 
one-fifth of all tabic patients have ocular paralyses. Partial or com- 
plete paralyses of the third nerve are the most common (54 per cent.), 
next comes paralysis of the abducens (33 per cent.), and of the superior 
oblique (8 per cent.), while in 5 per cent, all the exterior muscles are 
affected. In about one-quarter of the cases the optic nerve is simul- 
taneously affected, and in 10 per cent, there is facial paralysis. 

Tabic paralyses are apt to occur early in the disease. This is par- 
ticularly true of ptosis, which is very frequent indeed, and often, in 
fact, the only evidence of paralysis. * In any case, but especially when 
occurring early, tabic paralyses are usually incomplete, fluctuating and 
temporary. Often the paralysis affects only one or two muscles, and 
these incompletely; then, after lasting for a period varying from a 
few days to several months, and, after extending perhaps to other 
muscles in the same eye or its fellow, it disappears spontaneously. 
Recurrences are frequent either in the muscles originally affected or in 
others. The Argyll-Robertson pupil is usually found and the knee- 
jerk is usually absent. 

Paralyses Which develop later in the course of the disease are apt 

* Fraenkel thinks that in many of these cases of tabic ptosis, especially when 
very transient, there is no real paralysis, the patient failing to lift the lid simply 
because, owing to the accompanying anaesthesia, he cannot tell whether it is raised 
or not. To this condition may be given the name of pseudoptosis anwsthetica. 



THE EXTEA-OCULAR MUSCLES 229 

to be both more complete and more lasting, and some are absolutely 
permanent. A fair proportion of the cases of progressive, ultimately 
total ophthalmoplegia are tabic. * 

A tabes which has been preceded by syphilis or one which is com- 
bined with psychic symptoms is much more likely to cause eye-paralyses 
than an uncomplicated tabes. 

Most tabic paralyses are nuclear, although some are undoubtedly 
basal and even orbital in origin. Strtimpell thinks that the initial, 
transitory paralyses are especially apt to have a peripheral site. 

(&) Combined Lateral and Posterior Sclerosis. — This not infre- 
quently produces paralysis of the eye muscles. 

(c) Multiple Sclerosis. — Paralysis of the ocular muscles occurs 
very frequently in this disease (according to Uhthoff in from 17 to 
20 per cent, of the cases). Paralysis of the abducens (sometimes 
bilateral) is most frequent; trochlear paralysis is very rare. 

As in tabes, paralysis often occurs early, being sometimes the in- 
itial symptom of the disease; and also, as in tabes, the paralysis is 
apt to be incomplete and transitory. Indeed, a complete and permanent 
paralysis is very rare. 

An associated symptom of considerable diagnostic importance is 
nystagmus or pseudonystagmus (see Etiology of Nystagmus in Sec- 
tion V, page 278). 

The site of the paralysis is usually nuclear, but sometimes 
peripheral. 

(d) Syringomyelia. — Ocular paralyses, especially paralysis of the 
abducens, occur in about 11 per cent, of all cases of syringomyelia 
(Uhthoff). Here also true nystagmus is a frequent, associated symp- 
tom (Compare Chapter XV). 

(e) Acute Ascending Paralysis. — This not infrequently causes 
paralysis, due to change in the nuclei or the peripheral nerves, with, in 
some cases, degenerative changes in the muscles themselves. Bilateral 
abducens paralysis is most frequently observed ; next unilateral ab- 
ducens paralysis without or less often with paralysis of the third and 
fourth nerves. Paralysis of the third or fourth nerves without involve- 
ment of the abducens is rare (UhthofT"). (Compare Chapter XV.) 

(/) Progressive muscular dystrophy causes a primary orbital 
paralysis due to atrophic changes in the muscles themselves. 

(g) Other spinal diseases, very rarely causing ocular paralysis, are 
lateral sclerosis, Friedreich's ataxia, amyotrophic lateral sclerosis, pro- 
gressive muscular atrophy, poliomyelitis acuta, and myelitis. Chap. XV. 

10. General Paresis. — Paralyses of the ocular muscles may form a 



230 THE EYE AND NERVOUS SYSTEM 

prodromal symptom of general paresis or may develop after the psycho- 
sis has become well established. They are not very frequent and are 
usually transitory. In 1 to 2 per cent, of the cases, however, a chronic 
and progressive, ultimately total ophthalmoplegia develops (Chapter 
XII). 

11. Hysteria, Neurasthenia, and the Traumatic Neuroses very rarely in- 
deed produce paralysis of the individual muscles, causing rather paraly- 
sis or spasm of the associated parallel movements or of convergence. 
Apart from hysterical ptosis, which is probably in most cases referable 
to a spasm of the orbicularis, and which in other cases is probably a 
pseudo-paralysis due simply to anaesthesia of the lid (cf. Eraenkel, p. 
228, note), cases of paralyses of the abducens and of branches of the 
third nerve have been reported. Most of these are doubtful. In Sachs's 
case, for example, reported as an hysterical ophthalmoplegia, the 
paralysis apparently affected rather the associated movements than the 
muscles themselves. But genuine cases of abducens paralysis due to 
hysteria have been reported as by Duchenne, Leber, Parinaud, and 
Suker (See Chapter XVI). 

It need scarcely be added that diplopia occurring in hysteria is not, 
without further examination, to be taken as an evidence of paralysis. 

12. General Diseases causing paralysis are — 

(a) Syphilis. — This, especially in the tertiary stage, is by far the 
most frequent cause of paralysis. Apart from the post-syphilitic dis- 
eases, like tabes, general paresis, and lesions of the vessels (sclerosis 
and miliary aneurisms), all of which, as we have seen, so often produce 
ophthalmoplegias, syphilis acts directly to induce paralysis, by causing 
periostitis or gummy growth in the orbit; periostitis, meningitis, or 
gummy infiltrations at the base of the brain ; and gummata or degenera- 
tive processes in or near the nuclei. Probably at least half of the cases 
of ocular paralysis are due thus directly to syphilis. 

Paralysis of the exterior muscles, due to syphilis, is usually basal. 
The third nerve, in some or all of its branches, is the one most commonly 
affected, although any or all of the motor nerves of the eye may be 
attacked. The paralysis often develops very suddenly, and is fre- 
quently associated with or preceded by evidences of involvement of 
other cranial nerves, especially headache and anaesthesia or neuralgia 
along the branches of the fifth nerve. There may also be optic neuritis 
and either scotomata or contraction of the visual field, which latter may 
be more or less symmetrical, especially if, as often happens, the region 
of the chiasm is occupied by a syphilitic exudate. The paralysis when 
once developed is often progressive, but may be stationary or show 



THE EXTKA-OCULAK MUSCLES 231 

sudden variations, or even disappear altogether. It is not, however, 
so apt to be transitory, nor so apt to be limited to single muscles as in 
the case of tabic paralysis (Fournier). 

Congenital syphilis very rarely indeed causes paralysis. Cases, 
however, have been reported (Lawford, Zappert, and Schmidt-Rimpler 
cited by Bernheimer, who also reports a case; Cruchandeau). 

(b) Diphtheria. — About 5 or 6 per cent, of all cases of diphtheria 
are followed (usually in the course of three or four weeks) by a paralysis 
of accommodation, which is generally bilateral and is generally unac- 
companied by paralysis of the sphincter. 

Probably some 2.5 per cent. (Goodall, cited by Groenouw) of the 
cases of diphtheria are followed by paralysis of some of the exterior 
muscles of the eyeball. Much the most frequent form is paralysis of 
the abducens, which is often bilateral. This is frequently associated 
with paralysis of the accommodation. Complete paralysis of the third 
nerve is very rare (two of Tellais's cases and one of Parkinson's cited by 
Groenouw), and partial third-nerve paralysis is uncommon. 

Cases of trochlear paralysis have been described by Graefe and 
Krause. Uhthoff and others report a number of cases in which all the 
exterior muscles of both eyeballs (mostly without involvement of the 
accommodation) have been attacked. 

Other paralyses, especially of the soft palate, and not infrequently 
of the body-muscles (hemiplegia and paraplegia) may occur at the same 
time with the paralysis of the ocular muscles. 

Paralysis of the exterior muscles of the eyes is especially apt to fol- 
low severe cases of diphtheria, while paralysis of accommodation may 
occur in connection with very light forms of the disease (Groenouw). 

The paralysis in some epidemics appears and disappears very 
suddenly, leaving one muscle to attack another (Pagenstecher, 
cited by Groenouw). In almost all cases the paralysis ultimately 
disappears altogether. Miihsam (cited by Groenouw), however 
reports one case of permanent paralysis of acommodation ; and Mor- 
vat and I have reported cases of permanent paralysis of the exterior 
muscles. 

In at least three of the reported cases (Groenouw, Tellais), death 
ensued soon after the development of the paralysis. 

The lesion causing the paralysis has been variously ascribed to 
capillary ha?morrhages in the nerve-nuclei, disease of the blood-vessels 
supplying the nuclei or nerves, basal or orbital neuritis, and inflam- 
matory changes in the muscles themselves (Groenouw). 

(c) Influenza often causes a paralysis of accommodation, and pa- 



232 



THE EYE AND NEKVOUS SYSTEM 



ralyses of the exterior muscles are not very infrequent. The abducens 
is especially apt to be affected, although paralysis of some or all of the 
muscles supplied by the third nerve is also observed. Isolated trochlear 
paralysis is very rare. In some instances there is paralysis, complete 
or partial, of all the muscles moving the eyeball. 

The paralysis of the accommodation is usually bilateral, the paraly- 
sis of the exterior muscles unilateral, although bilateral paralysis of 
the abducens (Bernheimer), the trochlear (PfLiiger), and the third 
nerve have been reported. 

In these paralyses recovery is the rule. In some cases, however, 
especially when the exterior muscles are involved, the paralysis remains 
indefinitely. 

The lesions causing the paralysis are supposed to be neuritis, nu- 
clear or basal hemorrhages, circumventricular hemorrhages with the 
picture of a polioencephalitis superior (page 227), and a true encephali- 
tis. Influenza may also cause paralysis by inducing an orbital cellu- 
litis or an inflammation of Tenon's capsule. 

(d) Tuberculosis may cause paralysis by inciting a tuberculous 
meningitis (page 227). Otherwise, paralysis is very rarely indeed the 
result of tuberculosis, although now and then it may be induced by 
tuberculous foci situated at the foramina of exit of the nerves, or at the 
base of the skull or in the brain itself. The paralysis is usually basal 
or foraminal, very rarely nuclear. 

(e) Rheumatism. — Acute articular rheumatism occasionally pro- 
duces paralysis, mostly as a result of the metastatic processes (menin- 
gitis, orbital cellulitis, tenonitis) to which it gives rise. 

Much more frequent are certain paralyses to which the name rheu- 
matic is usually applied ; occurring generally as a result of exposure to 
cold, and especially in those who are the subjects of chronic rheuma- 
tism. The paralysis usually develops suddenly and is often associated 
with various rheumatic manifestations, with neuralgic pains along the 
course of the fifth nerve, and sometimes with paralysis of the facial. 
The abducens is most commonly affected, but paralyses of the other 
nerves, complete or partial, are not infrequent. 

Such paralysis is usually quite amenable to treatment with salicy- 
lates and by diaphoresis. 

(/) Herpes zoster ophthalmicus, especially the forms that are asso- 
ciated with fever or are due to toxic conditions, often cause paralysis. 
This usually affects isolated muscles supplied by the third nerve, 
although sometimes all the branches of the latter are attacked. The 
fourth and sixth nerves are rarely affected. 



THE EXTKA-OCULAR MUSCLES 233 

(g) Typhoid Fever. — Paralysis of any of the motor nerves of the 
eye may develop in typhoid fever, usually after the second week but 
sometimes earlier. They are ascribed to complicating affections of the 
brain or meninges or to a peripheral neuritis (Groenouw), but may 
perhaps also be due to hemorrhage. They are sometimes transient but 
may be permanent. 

(h) Cerebrospinal meningitis (page 227). 

(i) Malaria very rarely causes an oculo-motor or an abducens paraly- 
sis, which sometimes recurs periodically with the fever and which dis- 
appears under the use of quinine. 

(/) Gerliers disease (a peculiar disease endemic in Switzerland, 
marked by recurrent attacks of vertigo and paresis of the extremities) 
often produces ptosis and paralysis of the abducens or of the individual 
branches of the third nerve. A somewhat similar, but often fatal, affec- 
tion is hubi-sagari, endemic in Japan. In this, too, ptosis and other 
ocular paralyses are frequent. 

(h) Beriberi often produces paralyses which are usually transient. 

(I) Other infectious diseases occasionally producing paralysis are 
pneumonia (Alt, Aldrich) ; tetanus and cephalic tetanus; Weil's dis- 
ease (Groenouw) ; mumps; whooping-cough (Gowring, Batten); 
erysipelas and pysemic states. These probably act mainly by their 
secondary effects (meningitis, orbital cellulitis, hemorrhages due to 
convulsions). 

13. General conditions not essentially infectious are — 

(a) Diabetes. — In some dozen recorded cases, paralysis of the eye 
muscles has been caused by diabetes mellitus, and in one or two in- 
stances by diabetes insipidus. In the latter case there was probably a 
lesion about the fourth ventricle, causing both the polyuria and the 
paralysis. 

(b) Renal Disease. — A few cases of paralysis due to this cause have 
been reported by Dunn, and by other authorities (Knies, Bernheimer). 
They are in all probability due to hemorrhage. They appear usually 
to indicate a fatal issue. 

(c) Exophthalmic goitre occasionally causes paralysis of the ab- 
ducens and less often of the exterior muscles supplied by the third 
nerve. 

(d) Acromegaly. — Apart from the rather frequent cases of paraly- 
sis produced by the tumors of the hypophysis in acromegaly (page 228), 
paralysis is sometimes apparently due to some influence that this disease 
exerts upon the general condition (Bernheimer). The abducens and 
trochlear nerves are rarely, if ever, affected. 



234 THE EYE AND NERVOUS SYSTEM 

(e) Auto-intoxications from the digestive tract very rarely cause 
paralysis of the exterior muscles. 

(/) Profuse Hemorrhage. — Two cases of bilateral abducens paraly- 
sis due to this cause are recorded by Neuburger. 

14. Poisons. — There are a number of poisons which produce paraly- 
sis of the interior muscles of the eye (sphincter and ciliary muscle). 
For these, called cycloplegics, reference must be had to Chapter VI. 
Paralysis of the exterior muscles, on the other hand, is rarely caused 
by j^oisons. 

(a) Alcohol. — Chronic alcoholism in a few cases (about 0.5 per 
cent., according to Uhthoff) produces paralysis. Bilateral paralysis 
(usually incomplete) of the abducens is the most common form. This 
seems to be due to a nuclear lesion. 

Alcoholism also appears to play an important part in the genesis of 
polioencephalitis superior, which, as already stated (page 227) is asso- 
ciated with a progressive, ultimately total ophthalmoplegia. 

(b) Sulphuric acid may in very rare instances cause paralysis 
by producing polioencephalitis superior. 

(c) Gelsemium often causes ptosis and diplopia. 

(d) Other poisons occasionally producing paralysis of the exterior 
muscles are tobacco (cases not certain, although Wilkinson's case seems 
authentic); atropine (Baas); snake-venom; lead; carbon monoxide 
(illuminating gas) ; fungi; and decayed meat, fish, oysters, etc. (pto- 
maine-poisoning). In ptomaine-poisoning ptosis is comparatively fre- 
quent. 

15. Diseases of the nose, and especially of its accessory sinuses, produce 
paralysis, mainly by mechanical pressure, but sometimes apparently 
because of resorption of pent-up purulent matter. The same is ap- 
parently true of some cases of otitis , which produce paralysis, not by the 
pressure due to an otitic abscess (page 227), but by the resorption of 
infective material (Bernheimer). 

Diagnosis of Cause. — 1 . Most Frequent Causes. — Of the long list of 
causes above given, the ones most frequently encountered are syphilis, 
tabes, multiple sclerosis, exposure to cold (rheumatic paralyses), trau- 
matism, meningitis, tumors and abscess of the brain, arteriosclerosis 
and aneurisms (producing pressure or hemorrhage). Less frequent 
are general paresis, diphtheria, influenza, alcoholism (especially as in- 
ducing polio-encephalitis). 

A suddenly developing paralysis is usually due to exposure to cold, 
traumatism, hemorrhage, embolism, or thrombosis. In some cases of 
diphtheria the paralysis also appears and disappears suddenly. 



THE EXTRA-OCULAR MUSCLES 235 

Incomplete and temporary , more or less fluctuating, paralyses are the 
special characteristic of tabes (particularly in the early stages) general 
paresis, and multiple sclerosis. Post-diphtherial and rheumatic paraly- 
ses are also usually temporary, and traumatic paralyses often so. 

2. Abducens Paralysis is frequently caused by syphilis, tabes, and 
tuberculous meningitis, although these conditions still more often pro- 
duce oculo-motor paralysis. On the other hand, traumatism, exposure 
to cold, cerebrospinal meningitis, multiple sclerosis, syringomyelia, 
acute ascending paralysis, acute bulbar paralysis, pons lesions, diph- 
theria, influenza, and alcoholism much more often cause paralysis of 
the abducens than of the exterior muscles supplied by the third nerve. 
Congenital paralysis also often affects the abducens. In some of the 
conditions here noted, especially acute ascending paralysis, diphtheria, 
and alcoholism, both abducens nerves are often affected simultaneously. 

3. More or less complete paralysis of the third nerve is very often due 
to syphilis. Other conditions particularly apt to cause this form of 
paralysis are tumors and abscesses of the temporal lobe, tumors at the 
base of the brain (especially the hypophysis tumors of acromegaly), 
and aneurism of the internal carotid. Complete paralysis of the third 
nerve is not common in tabes, and bilateral complete third-nerve paraly- 
sis is hardly ever tabic (Uhthoff ). 

4. Paralysis of isolated branches of the third nerve is found especially 
in tabes and herpes zoster, but is also often due to syphilis, and may be 
caused hj traumatism or be congenital. 

5. One form of isolated paralysis of the third nerve, — namely, ptosis, 
— is of special diagnostic importance. 

Ptosis without paralysis of any of the muscles moving the eyeball 
occurs most often under the following conditions : 

(a) As a congenital state. Here usually bilateral, often incom- 
plete, and always permanent. 

(b) As a condition developing without assignable cause in old age. 
Always bilateral, gradual in development, and unassociated with any 
•other evidence of nerve trouble. 

(c) In tabes. Frequent, especially in initial stage of tabes. Usu- 
ally incomplete and transitory. May be a pseudoptosis (page 228 note). 

(d) In multiple sclerosis. Rare. 

(e) As an initial symptom of chronic progressive ophthalmoplegia. 
(/) In syphilis. Rare. 

(g) In cerebral softening (embolic or traumatic). Quite frequent. 

(h) In cerebral abscess, particularly otitic abscess of the temporal 

lobe on the same side as the ptosis. Here often associated with paresis 



236 THE EYE AND NERVOUS SYSTEM 

of the opposite side of the face and arm, sometimes of the leg. The 
same combination may occur in tumors of the brain. 

(i) In cerebrospinal meningitis. Not uncommon. 

(j) In cerebral hemorrhage. Occasionally. 

(k) Traumatism affecting the lid or levator directly, or occasionally 
traumatism producing intracranial lesion (even fracture of the 
base). 

(I) As evidence of a cortical lesion. (Usually in or near the angular 
gyrus on the opposite side to the ptosis). Rare. 

(m) In hysteria. Usually bilateral. 

(n) In ptomaine-poisoning. Associated with mydriasis and paraly- 
sis of accommodation. 

(o) A slight degree of ptosis is produced by paralysis of the superior 
rectus, provided this is not due to division of the tendon, a lesion which 
causes retraction of the lid (page 226). 

Ptosis with more or less involvement of the extra-ocular muscles 
supplied by the third nerve is found often in tabes, syphilis, cerebral 
softening, cerebral abscess, tumors in various parts of the brain, men- 
ingitis (especially tuberculosis, Gerlier's disease, polioencephalitis 
superior, and as a result of traumatism. 

A bilateral incomplete ptosis, which, slight in the morning, in- 
creases markedly in the evening, is characteristic of myasthenia gravis. 

Ptosis associated with abducens paralysis (with or less frequently 
without involvement of the extra-ocular muscles supplied by the third 
nerve) is very frequent in tabes. 

In using ptosis as a diagnostic sign we must be careful to exclude 
those conditions in which a drooping of the lid obtains but is not due 
to third-nerve paralysis. Such conditions are — 

(a) Sympathetic Ptosis. — In this the palpebral fissure is moder- 
ately contracted, but as much by raising of the lower lid as by drooping 
of the other. Repeated instillations of cocaine will not widen the 
palpebral fissure as they will in a third-nerve ptosis. There is usually 
miosis, and the pupil is undilatable by cocaine. There may be hyperi- 
drosis or anidrosis with redness or pallor of the face on the side of the 
lesion. This kind of ptosis is quite frequent in tabes. 

(b) Pseudoptosis from spasm of the orbicularis (spastic ptosis) 
occurs in irritative conditions of the eye, and is the usual form of ptosis 
in hysteria. Perhaps also the ptosis observed in pons lesions is due to 
this cause (Schoeler). In this form, as in sympathetic ptosis, both 
lids, the lower as well as the upper, take part in producing contraction 
of the palpebral fissure. 



THE EXTRA-OCULAK MUSCLES 237 

(c) Drooping of the lid from mere excess of weight or mechanical 
causes as in tarsitis syphilitica, trachoma, marked oedema, etc. 

(d) The pseudoptosis ancesthetica (Fraenkel, page 229, note). 

(e) The slight drooping of the lid produced by an advancement of 
the superior rectus. This operation, by carrying the tendon forward, 
draws forward also the superior palpebral band and with it the upper 
lid, which is thus depressed one or two millimetres. Similarly, advance- 
ment of the inferior rectus causes a quite perceptible elevation of the 
lower lid. 

6. Paralysis of the exterior muscles supplied by the third nerve without 
involvement of the fourth or sixth nerves is rare in acute ascending 
paralysis, syringomyelia, acute bulbar paralysis, diphtheria, influenza, 
and alcoholism. 

7. Isolated trochlear paralysis is most often due to syphilis, tabes, 
meningitis, sometimes to traumatism (especially injuries about the 
trochlea). It is seldom the result of diphtheria, influenza, or pons 
lesions, and is very rarely caused by multiple sclerosis. 

8. Simultaneous paralysis of the third and sixth nerves on the same side 
ocurs in fractures of the base, especially one which traverses the petrous 
bone and splits off the clinoid process. 

9. Total ophthalmoplegia, — i.e, the condition in which all three of the 
motor nerves of the eyes are paralyzed, — is most frequently due to 
syphilis, tabes (especially the later stages), orbital disease, and trau- 
matism. It is very rare in general paresis, diphtheria, and influenza. 

10. Ophthalmoplegia exterior occurs often in syphilis, also in tabes, 
and various basal and nuclear diseases. It is usually bilateral in 
polioencephalitis superior and the chronic progressive ophthalmoplegia 
of Graefe (page 242). 

Diagnosis of Site. — The following distinctive signs often obtain 
in cases of Orbital paralysis. 

1. The paralysis is usually unilateral. 

2. It frequently affects a single muscle. 

3. The interior muscles are not usually affected, except in the case 
of a paralytic mydriasis produced by a blow upon the eye itself, or a 
sympathetic miosis produced by injury to the orbit. 

4. In traumatic cases, particularly, there may be enophthalmos 
often associated with a sympathetic miosis (page 226). 

5. In tumors or inflammatory processes in the orbit there is often 
exophthalmos or a lateral displacement of the eye. 

6. Other evidences of orbital lesion are often present, such as scars 
in the lids or the margin of the orbit, depressed bone, orbital swelling 



238 THE EYE AND NERVOUS SYSTEM 

appreciable by palpation, deep pain elicited by pressure made into the 
orbit, chemosis, evidences of disease of the accessory sinuses. 

It must, however, be remembered that the presence of these evi- 
dences of orbital disease is not proof positive that the paralysis itself is 
orbital in site. Thus : 

(a) Exophthalmos may indicate a paralysis due to aneurism of the 
internal carotid or a thrombosis of the cavernous sinus. 

(6) Paralysis may occur with shot or stab wounds of the orbit be- 
cause the skull has been penetrated and the nerves injured at the base. 

(c) Paralysis occurring in an orbital cellulitis or orbital tumors 
may be caused by an extension of the morbid process backward into 
the cranial cavity and involvement of the nerves at the base. 

(d) Paralysis associated with marked evidences of orbital disease 
may, especially if syphilitic, be due to an intracranial process which 
after affecting the nerves at the base extends into the orbit. This is 
particularly apt to be the case if the process started by involving 
isolated muscles, especially those supplied by the third nerve. 

A total oculo-motor paralysis with completely intact fourth and 
sixth nerves is rarely, if ever, orbital (Wilbrand and Saenger). 

On the other hand, paralysis of all three motor nerves of the eye 
without involvement of other cranial nerves except the first branch of 
the fifth is almost certainly orbital or foraminal in origin. 

The following points are useful in diagnosticating a Basal paralysis. 

1. Paralysis of the other cranial nerves are often present (see next 
section on Affections of Other Nerves). 

2. In traumatic paralysis there are often evidences of fracture of 
the base (discharge of cerebrospinal fluid, bleeding from the ears). 
There may also be blood in the fluid obtained by lumbar puncture. 

3. In paralysis due to meningitis there are retraction of the head, 
fever, delirium, and severe headache, and leucocytes are present in the 
fluid obtained by lumbar puncture. 

4. In paralysis due to otitis there may be found, besides the evi- 
dences of ear disease with the accompanying deafness and vertigo, the 
signs of an abscess in the temporal lobe (optic neuritis, crossed facial 
paralysis and hemiplegia, aphasia, word-deafness, mind-blindness, 
homonymous hemianopsia). Similar signs would be found in temporo- 
sphenoidal tumors. 

5. Complete or nearly complete third-nerve paralysis, especially if 
bilateral, is usually basal or foraminal. 

6. Recurrent ophthalmoplegia (ophthalmoplegic migraine — see 
Chap. XVII) is usually due to basal lesions. 



THE EXTRA-OCULAR MUSCLES 239 

7. It was formerly thought that an ophthalmoplegia exterior and 
isolated paralyses of muscles supplied by the third nerve must be nu- 
clear, but in a number of cases these conditions have been shown to be 
due to basal lesions (UhthofT, Bernheimer, Salomonsohn, Hochwart). 

Many of the cases of what were thought to be nuclear paralysis are 
really fascicular, and not a few of the rest are really basal. If, as is 
generally the case in tabes and multiple sclerosis, the paralysis is evi- 
dently due to central causes, it is probably fascicular if more than one 
nerve is involved. This rule, however, is not without its exceptions, as 
several instances of multiple nuclear paralyses have been noted (Bern- 
heimer). 

The diagnosis of paralysis due to a lesipn of the pons is based on 
the presence of the following: 

1. Paralysis of one (sometimes both) abducens nerves, or more fre- 
quently a paralysis of conjugate movement to the right or left (pages 
221 and 270). 

2. Optic neuritis (in 25 per cent, of the cases). 

3. Associated paralysis of the fifth, seventh (very frequent), eighth, 
or twelfth nerves. 

4. Ptosis (in some cases). 

5. Paralysis or paresis on the opposite side of the body. Very 
common. Sometimes also there are convulsive movements or general 
convulsions and sensory disturbances. 

Affections of Other Nerves as Determining the Diagnosis. 

1. Olfactory Nerve. — Paralysis of this, causing anosmia, occurs in 
paralyses associated with nasal disease and in tabes. 

2. Optic Nerve. — An optic neuritis associated with paralysis of the 
eye muscles suggests, in most cases, the probability that the paralysis is 
due to tumor or abscess of the brain or to syphilitic or tuberculous 
meningitis. Less often it occurs in paralysis due to multiple sclerosis 
or to morbid processes in the orbit, implicating the optic nerve. 

Total optic-nerve atrophy occurs in at least 25 per cent, of paralyses 
due to tabes and is also common in paralysis resulting from orbital in- 
flammation or tumors (then usually associated with exophthalmos), 
traumatism (especially fractures at the apex of the orbit), and perios- 
titis of the sphenoidal fissure. A usually partial (temporal), but some- 
times total optic atrophy often accompanies the motor paralyses pro- 
duced by multiple sclerosis. 

Homonymous hemianopsia occurs in paralysis due to basal lesions 
affecting the optic tract, brain tumors, and otitic abscesses of the tem- 
poro-sphenoidal lobe. 



240 THE EYE AND NEKVOUS SYSTEM 

Bitemporal hemianopsia may occur in paralyses due to chiasm 
lesion (especially acromegaly). 

Concentric contraction of the visual field occurs in tabic paralyses 
and irregular contraction or a central scotoma often in multiple 
sclerosis. 

3. Trigeminus. — Paralysis of all branches of the fifth combined with 
paralysis of the third (less often of the fourth or sixth) nerves occur 
especially in syphilis, in tabes, in tumors of the base, and in aneurism of 
the internal carotid. 

Paralysis of the first branch of the fifth (with perhaps keratitis neu- 
roparalytica) combined with paralysis of the third and with optic-nerve 
atrophy indicates disease (syphilitic periostitis) in the sphenoidal 
fissure. 

Paralysis of the fifth combined with paralysis of the abducens 
occurs especially in pons lesions and in syringomyelia. 

Neuralgia of the fifth occurs often in syphilitic and rheumatic 
paralysis. 

4. Facial. — Paralysis of the facial occurs conjointly with paralysis 
of the eye muscles as a result of 

(a) Tabes (in about 10 per cent, of tabic paralyses). 

(b) Otitis and particularly otitic abscess of the temporo-sphenoidal 
lobe, in which case the most usual combination is a paralysis (generally 
partial) of the third nerve on the side of the abscess and paralysis of 
the opposite facial. Similar conditions may be produced by a tumor 
situated in the frontal or temporal lobes. 

(c) Exposure to cold. Here the most frequent combination is 
paralysis of facial and abducens. 

(d) Traumatism, especially fracture through the petrous bone im- 
plicating the facial and the abducens or sometimes the facial and the 
third nerve together. 

(e) Pons lesions. Paralysis of the facial (usually the upper facial 
being exempt) occurs in about two- thirds of the cases of pons disease, 
and is then very frequently combined with paralysis of the abducens. 

(/) Acute ascending paralysis (not infrequent) and occasionally 
also in other spinal diseases (myelitis, etc.). The most usual combina- 
tion is paralysis of the facial and abducens. 

5. Auditory. — Paralysis of the eighth nerve (nerve deafness, may 
occur combined with eye paralyses in otitis, fracture of the petrous 
bone, syphilis, tumors of the auditory nerve, and pons lesions (in the 
latter case in conjunction with abducens paralysis). 

6. Hypoglossal. — This is very often involved in conjunction with the 



THE EXTKA-OCULAE MUSCLES 241 

abducens of the same or the opposite side in pons lesions ; sometimes 
also in lesions situated in the posterior cranial fossa near the middle 
line. 

7. Hemiplegia. — Paralysis of the third nerve with complete hemi- 
plegia of the opposite side of the body indicates usually either a lesion 
in the peduncle, or an otitic abscess in the temporo-sphenoidal lobe (in 
the latter case often also combined with paralysis of the opposite facial), 
or a tumor in the frontal or temporal lobe. 

Paralysis of the sixth nerve with hemiplegia of the opposite side of 
the body indicates a lesion of the pons situated on the same side as the 
paralyzed abducens. 

Course and Prognosis of Paralyses. — As already stated, some 
paralyses — especially those due to direct effects of traumatism and to 
hemorrhage, embolism, thrombosis, and exposure to cold — begin sud- 
denly. This is also the case with many diphtherial and some syphilitic 
and tabic paralyses. 

In other cases the paralysis develops slowly — sometimes very slowly 
indeed, taking many years before it reaches its height. 

The duration of a paralysis varies from a few hours to a lifetime. 
A similar variation is seen in the completeness of the paralysis, which 
may range from a slight restriction of movement of a single muscle to 
a total ophthalmoplegia producing absolute immobility of the eyeball. 

Transitory paralyses are the rule in tabes and multiple sclerosis. 
The paralysis is also usually temporary when due to diphtheria, ex- 
posure to cold, beriberi, and ptomaine-poisoning; and often so when 
caused by general paresis and influenza. Traumatic paralysis is fre- 
quently temporary if due to the pressure of effused blood, which can 
undergo absorption, to division of a tendon which subsequently becomes 
re-attachecl, or to some similar remediable condition. 

Paralyses which occur early in the course of a central nervous dis- 
ease — particularly tabes, multiple sclerosis, and syringomyelia — are apt 
to be less severe and less permanent than those developing in the later 
stages. The same is probably true, although perhaps not so markedly, of 
syphilitic paralyses. In syphilis, however, the paralyses usually occur 
late in the disease, and in any case, although they vary greatly in 
intensity and duration, are generally more lasting, more complete, and 
more likely to involve a number of muscles than are the paralyses found 
in tabes and multiple sclerosis. 

Congenital paralyses usually remain unchanged through life. An 
exception seems to be strabismus fixus (page 224), which, even when 
congenital in origin, may be aggravated by consecutive changes in adult 
life. _ 



242 THE EYE AND NEKVOUS SYSTEM 

Traumatic paralyses, too, are apt to remain stationary, especially if 
complete, and the same is true of other paralyses when a cause that has 
ceased to act has produced complete destruction of a nerve or muscle. 

In some cases a paralysis shows a sudden and great increase. This 
may occur in incomplete traumatic paralyses very soon after the injury, 
and is then referable to a sudden giving way of the adhesions or of a 
severed but re-attached tendon. But it may also occur, and without 
apparent cause, in paralyses whether traumatic or not, which have 
remained stationary for a long time. 

In some cases an apparent sudden increase in a paralysis is pro- 
duced by the fact that the patient fixes with the paretic eye, allowing 
the sound eye to deviate. This, without really changing the muscular 
conditions, causes a great increase in the deviation (page 215). 

If, as sometimes happens (page 217), fixation is performed with 
each eye alternately, the deviation will show a sudden great increase 
when the paretic eye is used, and it will seem, as the patient shifts from 
one eye to the other, as if the paralysis was undergoing sudden and 
great changes in amount. 

In other cases still, a paralysis tends to advance gradually and either 
continuously or discontinuously {progressive paralysis). Cases of this 
sort in which ultimately nearly all the eye muscles are involved, occur 
in syphilis and tabes. 

A peculiar form of progressive paralysis is the rare chronic progres- 
sive ophthalmoplegia of Graefe. This is an extremely chronic affection, 
which often begins in childhood and affects very gradually one muscle 
after another until after many years the picture of a more or less com- 
plete ophthalmoplegia exterior is produced. It may be arrested in any 
stage of its course. In any case the interior muscles are exempt, and, 
aside from the ophthalmoplegia, no sign of other disease of the nervous 
system subsequently develops. The affection is always bilateral. 

A paralysis, however caused, may disappear suddenly. Much more 
frequently the recovery is gradual, and often enough it is incomplete, 
a more or less marked paresis — sometimes only a very slight insuffi- 
ciency — of one or more muscles remaining indefinitely. 

Even when the recovery is complete, recurrences may take place. 
Such recurrences may affect the muscle originally involved, or some 
other muscle in the same eye, or one or more muscles in the fellow-eye. 
They are observed especially in tabes and in syphilis, but may also 
occur in paralysis resulting from exposure to cold and those caused by 
tumors and aneurisms, syringomyelia, chronic meningitis, multiple 
neuritis, chronic progressive ophthalmoplegia, and other causes. 

Paralyses which disappear and recur at short, regular intervals 



THE EXTRA-OCULAR MUSCLES 243 

may occasionally develop in connection with malaria and perhaps in 
other conditions. Very peculiar are the cases described by Salzmann 
(cfted by Wilbrand and Saenger), and by Axenfeld and Schiirenberg, 
in which, with an ocnlo-motor paralysis, there was rhythmical return of 
power — indeed an actual spasm — in the levator and in the interior 
muscles every minute or so. Such conditions are attributed to periodi- 
cal changes in the blood-supply of the oculo-motor nucleus (Fuchs), 
or, with more plausibility, to the intermittent pressure of a self -empty- 
ing angioma on the trunk of the nerve at the base of the brain (Axen- 
feld and Schiirenberg). 

A peculiar variety of recurrent paralysis usually affecting the third, 
but sometimes the fourth or sixth nerves, is the so-called ophthalmo- 
plegic migraine (Chapter XVII). Another affection characterized by 
intermittent recurrent paralyses is Gerlier's disease (Chapter X). 

In acquired paralysis, particularly when it is complete and when 
it has lasted a long time, a consecutive contracture of the antagonist to the 
paralyzed muscle commonly develops. Thus, in paralysis of the ex- 
ternus, the interims becomes contractured, causing a strong deviation 
of the eye inward. In paralysis of the internus, contracture takes place 
in the externus; in paralysis of the superior rectus the contracture 
affects sometimes the inferior rectus, sometimes the superior oblique; 
and in paralysis of the inferior rectus, it affects either the superior 
rectus or the inferior oblique. 

The effect of the contracture is to extend the deviation and render 
it comitant. That is, a deviation which is originally confined to one 
portion of the field of fixation becomes spread over all portions. Thus, 
in paralysis of the right abducens, there is originally a marked inward 
deviation which increases progressively as the eyes are carried to the 
right, and diminishes to zero as the eyes are carried to the left. Then 
a consecutive contracture of the right internus takes place, and now the 
right eye being pulled strongly inward by this muscle, shows a marked 
inward deviation in the primary position and also when turned to the 
left. Ultimately such a case cannot be differentiated from a comitant 
strabismus convergens of the right eye. 

So, too, in a paralysis of the right superior rectus the right eye at 
first simply drops below the left and does so only when the patient 
looks up, while when he looks down the two eyes are on a level. After- 
wards a contracture of the inferior rectus or the superior oblique takes 
place, and then the right eye is forcibly carried down, so that it is 
below the left, not only when the eyes are directed up, but also when 
they are in the primary position and when they are directed down. The 



244 THE EYE AKD NERVOUS SYSTEM 

condition is thus transformed from a non-comitant deviation into a 
more or less comitant vertical squint (strabismus deorsumvergens) . 
Similarly a paralysis of the superior oblique or the inferior rectus may 
through contracture of the opposing elevators produce a comitant strabis- 
mus sursumvergens. 

While contracture of the opponent is regularly present in old ac- 
quired paralyses, it is as regularly absent in congenital paralysis. Both 
rules, however, have their exceptions (A. Graefe). 

In some cases the paralysis itself may disappear and the contracture 
persist, leaving a pronounced spastic deviation (A. Graefe). 

More or less variable spasm may occur in the opponents of the par- 
alyzed muscle. Thus, in paralysis of the inferior rectus, a condition 
which is associated mainly with diplopia in looking down, a transient 
spasm of the superior rectus or inferior oblique may occur, causing 
sudden changes in the degree of deviation and producing diplopia in 
looking up, which diplopia will vary in amount from day to day. In 
my belief such spasm is the usual forerunner of a permanent contrac- 
ture and is indeed the cause of it. 

Treatment of Paralysis. — In treating a paralysis the first and fore- 
most indication is to remove its cause. Accordingly, in paralyses due 
to syphilis and the parasyphilitic diseases (tabes, general paresis) we 
use large doses of the iodides and mercury. In syphilis, where the 
paralyses often occur "late in the disease and indicate a deep-seated lesion, 
which may be the immediate precursor of even more serious changes 
in the nervous system, treatment should be pushed very vigorously and 
kept up for many months. The earlier such thorough-going treatment 
is initiated, the greater the prospect both of relieving the paralysis 
itself and of preventing the development of other serious manifestations 
later on. 

In paralyses due to exposure to cold, treatment with diaphoretics 
and salicylates is indicated and is usually successful. 

In paralyses resulting from diphtheria, influenza, hysteria, and 
neurasthenia, tonic treatment should be employed. Use of the eyes for 
near work should be restricted or forbidden for a time, at least, so as 
to give the central neurons a chance to regain their full activity. 

Strychnine has been recommended in paralysis, but seems of doubt- 
ful value except as a general tonic. 

Electricity has been used with success by Benedikt (cited by Beard 
and Rockwell), by Roy, and others. 

Passive traction (made with forceps under cocaine), exercises in 
moving the eye (as in following a moving object), and exercises in 






THE EXTEA-OCULAK MUSCLES 245 

overcoming diplopia, either with or without prisms, may be of use in 
partial paralyses and particularly as an adjuvant when the paralysis 
shows any tendency to recover. This treatment is much more effective 
in peripheral (and especially traumatic) paralyses than in those of 
central origin. 

Finally, if the paralysis has remained stationary for months, and 
there is no apparent prospect of a change taking place in it, an opera- 
tion may be done. This consists in 

(a) Advancement of the paretic muscle itself. Done especially in 
partial and in traumatic paralyses. 

(&) Advancement of another muscle in the same eye, especially of 
the superior rectus if the inferior oblique is paralyzed. 

(c) Tenotomy of the direct antagonist in the same eye, — e.g., of the 
right internus when the right externus is paralyzed. Should be done 
only in case there is well-marked contracture of the direct antagonist. 

(d) Tenotomy of the associate muscle (page 194) in the fellow- 
eye, thus of the left internus, externus, inferior rectus, superior rectus, 
inferior oblique, and superior oblique, respectively, in the case of 
paralysis of the right externus, internus, superior oblique, inferior 
oblique, superior rectus, and inferior rectus. This operation affords 
very good results. 

In many cases a combination of several of these operations may be 
required. 

To obviate the diplopia due to the paralysis many patients find it 
advantageous to shut out the sight of one eye by putting a patch or a 
ground glass before it. Others find these devices intolerable. 

Conditions Allied to Paralysis. — Dyskinesis is the condition in 
which the muscles are not paretic and are, in fact, capable of contracting 
to the full normal extent, yet act sluggishly or with pain and difficulty. 
It may occur after any exhausting illness, as an evidence of neuras- 
thenia or hysteria, or as one of the symptoms of general muscular en- 
feeblement from any cause. In some cases it seems to occur without ob- 
vious cause and as an independent affection in otherwise healthy people. 

A peculiar ataxia of the eye-muscles sometimes, although very rarely, 
occurs in tabes (Uhthoff). If the atactic movements are associated 
(comitant) so that one eye acts precisely like the other, there will be 
no diplopia. In this case the condition is an ataxia rather of the ocular 
movements than of the muscles themselves, and may be regarded as a 
kind of irregular nystagmus. When the atactic movements differ in the 
two eyes, so that the eyes make dissociated movements, a peculiarly 
irregular and changeable diplopia will ensue. 



246 



THE EYE AND NEKVOUS SYSTEM 



Section III. 
SPASM OF THE INDIVIDUAL OCULAR MUSCLES. 

Symptoms. — Normally, when the eyes are carried in a given direc- 
tion, they move equally far and equally fast, because the two associate 
muscles (page 194) chiefly concerned in that special movement work 
precisely alike. If one of these muscles is underacting, the eye to which 
it is attached lags more and more behind the other in performing this 
special movement, and we have in consequence an increasing deviation, 
an increasing diplopia, and the other symptoms of paralysis already 
noted. Quite the same thing will happen if one of the associate muscles 
is overacting. In that case the affected eye will shoot further and further 
ahead of the other, and we will have again a deviation and a diplopia, 
increasing as the eyes are carried in the given direction. 

Accordingly, the evidences of a spasm or overaction are as follows : 

1. Excessive Movement. — The affected eye shoots beyond the other 
when the eyes are carried in a certain direction, and its total range of 
excursion in that direction is greater than that of the fellow-eye. 

2. The deviation thus produced, which can be made out by the 
screen and other tests, is greater if the sound eye is used for fixation 
than if the affected eye fixes, — i.e., the primary is greater than the 
secondary deviation. (Cf. remarks, page 198, 212). 

3. Diplopia, increasing as the eyes are carried in the direction of 
the field of action of the overacting muscles. This diplopia is precisely 
the same in character and in behavior as that which would be produced 
by a paralysis of the associate muscles in the fellow-eye, — i.e., the con- 



ditions will be 


as shown in the following table. 






CORRESPONDS TO 
PARALYSIS OF LEFT 




IN EITHER CASE 




SPASM OF RIGHT 


IF L. EYE 
FIXES R. 
DEVIATES 
GREATLY 


IF R. EYE 
FIXES L. 
DEVIATES 


DIPLOPIA IS 


DEVIATION AND 

DIPLOPIA IN- 
CREASE AS EYES 
ARE CARRIED 


External rectus. 


Internal rectus. 


Out. 


Out. 


Crossed. 


ToR. 


Internal rectus. 


External rectus. 


In. 


In. 


Homonymous. 


ToL. 


Superior rectus. 


Inferior oblique. 


Up. 


Down. 


Right.* 


Up and to R. 


Inferior oblique. 


Superior rectus. 


Up. 


Down. 


Right.* 


Up and to L. 


Inferior rectus. 


Superior oblique. 


Down. 


Up. 


Left.f 


Down and to R. 


Superior oblique. 


Inferior rectus. 


Down. 


Up. 


Left.| 


Down and to L. 



Note. — The conditions obtaining in spasm of the left eye (paralysis of the right) 
may be deduced by substituting right for left and vice versa. 

* I.e., vertical diplopia with the image of the right eye below. 
f I.e., vertical diplopia with the image of the left eye below. 



THE EXTRA-OCULAR MUSCLES 247 

4. False Projection. — If the projection test is made in the manner 
indicated on page 210 by having the patient close the good eye and try 
to put his finger on an object situated within the field of action of the 
affected muscle it will generally be found that in a case of spasm the 
patient undershoots his mark, just as in paralysis he overshoots it. 
Thus, a patient with a spasm of the right abducens who is trying to 
touch an object on his right, will put his finger to the left of it. The 
reason for this is that the patient underestimates the distance that the 
eye travels, since he is aware only of the effort that he is putting forth 
in order to look at the object, and with an overacting muscle this effort 
is less than normal. 

5. An apparent movement of objects looked at may be produced by 
the false projection, and this movement together with the diplopia may 
cause vertigo. 

As will be seen from the above, the appearances presented by a 
spasm of a muscle in one eye closely simulate those of a paralysis of 
the associate muscle in the other eye. Indeed, unless the objective 
signs of a paralysis, such as marked restriction of movement, are 
present, the diagnosis may be difficult. It can be facilitated by the 
following considerations : 

Poitits in Common. 
One eye moves faster and further than the other when both are carried in some 
one particular direction; and both this discrepancy between the position of the eyes 
.and also the diplopia, false projection, and vertigo become more and more pronounced, 
the further the eyes are carried in that direction. 

SPASM. PARALYSIS. 

Measured by the perimeter or tropometer, Measured by the perimeter or tropometer, 
the absolute excursion of the faster the absolute excursion of the faster 
moving eye in the given direction is moving eye is normal in all direc- 
greater than normal; that of the tions; that of the other eye is sub- 
other eye is normal. normal. 

The total excursion of the faster moving The total excursion of the faster moving 

eye in the given direction and in the eye is normal, that of the other eye 

direction opposite is greater than nor- is subnormal, — i.e., the field of fixa- 

mal, — i.e., the field of fixation is ex- tion of the latter is contracted in one 

cessively large in one of its diameters. of its diameters. 

Fixation is usually performed by the Fixation usually performed by the faster 

slower moving eye. moving eye. 

The amount of deviation may show great The amount of deviation rarely shows 

and sudden changes from time to time. these sudden changes. 

If false projection is present, it occurs False projection occurs when the slower 

when the faster moving eye is used for moving eye is used for fixation, and 

fixation (the other being closed), and the patient overshoots the mark he is 

the patient undershoots the mark he trying to touch. 
is trying to touch. 



248 THE EYE AND NERVOUS SYSTEM 

Etiology of Spasm. — Overaction of an ocular muscle may occur 
either because the muscle itself is excessively strong and overdeveloped 
{structural overaction), or because the tendon is inserted so close to 
the cornea that it exerts an undue leverage on the eye {insertional 
overaction). A marked example of insertional overaction is that pro- 
duced by the operation of advancement. 

True spasm is that form of overaction produced by excessive in- 
nervation. It may be either primary or secondary. 

Primary spasm of the ocular muscles is rare. A spastic, sometimes 
painful, contraction of one or more muscles occurs at times in menin- 
gitis and other irritative affections of the brain. It has also been ob- 
served as a result of reflex irritation due to decayed teeth (Eversbusch), 
or to middle-ear disease. Lucae found that artificial heightening of 
the labyrinthine pressure produces a divergent squint which he ascribes 
to spasm of the abducens. Tonic spasm also occurs in tetanus. Mac- 
kenzie says that in this disease sometimes all the eye muscles, interior as 
well as exterior, are thus affected. The eyeball in this case is fixed and 
motionless, so that the condition closely resembles a total ophthalmo- 
plegia, from which, indeed, it can be differentiated only by the fact that 
there are miosis and spasm of accommodation. 

Spasm also is occasionally due to hysteria. In most of the cases, 
however, in which the spasm is reported as affecting the eye muscles, it 
really affected some one of the co-ordinate movements of the eyes. In 
particular, the cases of spasm of the interni that have been reported are 
probably really cases of spasm of convergence. The same is probably 
true of Theobald's case of spastic squint occurring after influenza. 

The spasm that accompanies convulsive attacks is also usually a 
conjugate spasm, affecting the associated movements rather than a 
single muscle. Gowers, however, records several cases in which a 
single muscle, especially the external rectus, was the seat of a true epi- 
leptoid attack, — i.e., the muscle was suddenly seized with tonic or 
combined tonic and clonic spasms, which, after lasting a short time, 
disappeared, leaving the eye completely normal until the next attack. 
The seizures were sometimes preceded by an aura and were accom- 
panied by vertigo, diplopia, and some obscuration of consciousness. 

Gowers also states that slight clonic spasms of the eye muscles may 
occur in chorea. 

Desmarres, the celebrated ophthalmologist, suffered from a clonic 
spasm of the superior oblique, due to a lesion of the opposite cerebral 
hemisphere (Landolt, cited by A. Graefe). 

Secondary spasm is much more common than the primary. By sec- 



THE EXTKA-OCULAK MUSCLES 249 

ondary spasm we mean one which is the result of a paralysis of another 
muscle, either in the same eye or in its fellow. It occurs under the 
following forms: 

1. Secondary spasmodic deviation of the sound eye when the paretic 
eye fixes (page 215). This deviation, as already shown (page 217 and 
cf. Table, page 247), always appears under the guise of a spasm of 
the associate to the paralyzed muscle. 

2. Spasm of a synergic muscle in the same eye. If the right 
superior rectus is paralyzed, the extra nervous effort employed in keep- 
ing this eye in place will be imparted equally to its synergic muscle, 
the right inferior oblique. This muscle, in consequence, acts ex- 
cessively. In most cases the effect of this excessive action is not ap- 
parent, because the associate muscle in the left eye (the superior rectus) 
receives a similar excessive impulse. Sometimes, however, the im- 
pulse imparted to the oblique preponderates, and then we have the 
picture of a combined paralysis of the right superior rectus and spasm 
of the right inferior oblique. That is, we have a left diplopia which 
increases in looking up and to the right, and a right diplopia which 
increases up and to the left. This finding is quite like that which we 
get in a paralysis of both superior recti, and often differs only in the 
fact that the diplopia in the left upper field fluctuates markedly in 
amount from one examination to another. 

In an abducens paralysis we sometimes find a similar associated 
spasm of the obliques. This causes the eye to move by zig-zag jumps 
a little further out than the externus alone can carry it. If the eye 
is above or below the horizontal plane, the amount of additional move- 
ment thus obtained may be considerable. 

3. More or less irregular and varying spasm in the opponents of the 
paralyzed muscle (page 244). 

4. Permanent spastic contraction {contracture) of the opponent to 
the paralyzed muscle (page 243). As already noted, in this case the 
paralysis itself may disappear and the contracture remain. When this 
happens the picture presented will be that of spasm, pure and simple. 

Section IV. 

CONVERGENCE AND DIVERGENCE ANOMALIES. COM- 
ITANT HETEROPHORIA AND SQUINT. 

Nature of these Anomalies. — The remaining motor anomalies of 
the eye are not caused by weakness or overaction of the muscles, per s<\ 



250 THE EYE AND XERVOUS SYSTEM 

nor by any affection of the nerves supplying them. They are due 
to involvement of the centres or tracts connecting these nerves with 
one another and with the cortex, and consequently appear under the 
guise of either a deficiency or an excess of one of the co-ordinated 
movements of the eyes (convergence, divergence, or parallel move- 
ment). 

Convergence, divergence, and parallel movements are binocular 
acts, — i.e. , each eye takes a precisely equal part in executing them. 
Hence, if there is any excess or deficiency in the performance of these 
movements, owing to involvement of the centres governing them, the 
excess or the defect will show itself in each eye equally. Perversions 
of these functions are always binocular. 

When we look to the right we converge or diverge our eyes with 
the same facility as when we look to the left, and with nearly the 
same facility as when we look straight ahead. The deviation pro- 
duced by a convergence or divergence anomaly, therefore, will remain 
about the same, no matter how the eyes are turned, provided that the 
object of fixation is kept at the same distance from the eyes. But 
since generally we use our convergence strongly when looking at near 
objects and do not use it when looking at distance, the effect of a con- 
vergence anomaly in producing deviations will be much greater in near 
than in distant vision. The opposite will be the case with a divergence 
anomaly. Convergence and divergence anomalies then are comitant 
in that the deviation changes but little as the eyes are carried to the 
right or left, and are 'periodic in that the deviation due to a con- 
vergence anomaly increases, and that due to a divergence anomaly 
decreases, as the object of fixation approaches the eyes. 

Nearly all cases of periodic comitant heterophoria and squint are 
produced by an anomaly of convergence or divergence. Nearly all 
cases of continuous comitant squint are produced by an anomaly of 
convergence combined with one of divergence. 

Convergence-excess will produce an active, and divergence-insuffi- 
ciency a passive, convergence of the visual lines (esophoria or eso- 
tropia). Convergence-insufficiency and divergence-excess will pro- 
duce exophoria and exotropia. Ordinary comitant convergent squint 
(esotropia) is due to a convergence-excess combined with a divergence- 
insufficiency, and ordinary divergent squint (exotropia) to divergence- 
excess combined with convergence-insufficiency. 

Types of Periodic and Continuous Deviations. — The different 
types of periodic and continuous deviations thus produced have the 
following characters : 



THE EXTKA-OCULAR MUSCLES 

PEKIODIC HETEROPHORIA AND SQUINT 



251 



ESOPHORIA 
and CONVERGENT SQUINT 



Deviation for dis- 
tance by all tests 
(screen, parallax, 
Maddox rod, pho- 
rometer). 

Deviation for near 
(by all tests). 



Convergence 
point. 



Prism-convergence 
(adduction. ) 



Prism-divergence 
(abduction. ) 



Primary and second- 
ary screen devia- 
tion. 

Field of binocular 
fixation. 



Field of binocular 
single vision. 



CONVERGENCE- DIVERGENCE- 

EXCESS I INSUFFICIENCY 

I 



Orthophoria or 
slight esopho- 
ria. 



Esophoria 
marked (much 
more than for 
distance). 



Normal (or ab- 
normally 
close). 



Normal. 



Normal. 



Esophoria 
marked. 



Esophoria 
slight or none 
(decidedly 
less than for 
distance). 



Normal. 



Normal. 



EXOPHORIA 
and DIVERGENT SQUINT 



CONVERGENCE- DIVERGENCE- 

INSUFFICIENCY EXCESS 



Orthophoria or 
slight exopho- 
ria. 



Exophoria 
marked. 



Exophoria very I Exophoria de- 

marked(much cidedly less 

more than for than for dist- 
distance). ance. 



Remote (more i Normal, 
than 7 cm. 
from root of I 
nose). 



Low and ac- 
complished 
only with ef- 
fort and dif- 
ficulty. 

Normal. 



Normal. 



Abnormal ly 
great (over 9° 
of prism). 



Low less than 
(2°-3°) or 
negative (i.e., 
insuperable 
homonymous 
diplopia for 
distance). 

In all four types deviation of one eye behind the screen, measured 
with prisms, equals precisely that of the other eye (distinction 
from paretic deviations).* 

In all four types excursions of each eye normal in all directions. 
Neither eye lags behind the other when both are making lateral, 
vertical, or oblique movements (distinction from paretic devia- 
tions). 



Homonymous diplopia if present, 
not increasing (if anything, 
diminishing) as eyes are carried 
to right and left (distinction 
from paretic deviations). In- 
creases somewhat in looking 
down, diminishes in looking 
up. 



Crossed diplopia not increasing as 
eyes are carried to right and 
"left (distinction from paretic 
deviations). Increases some- 
what in looking up, diminishes 
in looking down. 



*In some few cases one eye does deviate more than the other when covered. This seems to 
occur in old cases of squint, in which secondary muscular changes have taken place in the squinting 
eye, so that the latter has in effect either a paretic or an overacting musculature. It also happens 
when, owing to excess of hyperopia in one eye or to some similar cause, the patient exerts more accom- 
modative effort when seeing with one eye than when seeing with the other. If, for instance, a man's 
right eye is much more hyperopic than his left he will accommodate strongly when the right fixes and 
the left is screened, and lience the left will then turn strongly in. Then when the screen is shifted to 
the right eye and the left fixes, less accommodative effort will be put forth, and the right eye will turn 
in but little or may even under certain conditions turn out (Graefe). 



252 



THE EYE AND KEKVOUS SYSTEM 

CONTINUOUS HETEROPHORIA AND SQUINT 



KSOPHORIA AND CONVERGENT SQUINT 

=convergence-excess+divergence- 
insufficiency. 



EXOPHORIA AND DIVERGENT SQUINT 

=convergence-insufficiency+diver- 
gence-excess. 



Deviation for dis- 
tance and near 
(by all tests) 
but especially 
the screen. 



Convergence near- 
point. 



Prism converg- 
ence (adduc- 
tion). 



Prism divergence 
(abduction). 



Prim ary and 
secondary screen 
deviation. 

Field of binocular 
fixation. 

Field of binocular 
single vision. 



Very marked esophoria or a 
marked convergent squint at all 
ranges (somewhat greater for 
near than for distance if the con- 
vergence-excess was the primary 
condition, and greater for dis- 
tance than for near, if the di- 
vergence-insufficiency was the 
primary condition). 

Normal or excessively close. 



If it can be elicited (i.e., if there 
is no suppression), normal or 
supernormal. 



Low. Usually (in case of squint, 
always) negative (i.e., there is 
homonymous diplopia at all 
ranges, so that no prism, base 
in, is overcome, or there is mon- 
ocular vision with suppression). 

As in periodic deviations. (See 
table preceding. ) 



As in periodic deviations. (See 
table preceding. ) 

As in periodic deviations. (See 
table preceding. ) 



Marked exophoria or marked di- 
vergent squint at all ranges 
(somewhat greater for near than 
for distance if the convergence- 
insufficiency was the primary 
condition, and greater for dis- 
tance than for near if the diver- 
gence-excess was the primary 
condition). 

Remote. In case of actual squint, 
none (patient may try to con- 
verge but cannot secure binoc- 
ular fixation at any distance). 

Low. In case of actual squint 
negative (i.e., there is either 
crossed diplopia at all ranges so 
that no prism is overcome, or 
there is monocular vision with 
suppression). 

If it can be elicited (i.e., if there 
is no suppression) it is much 
greater than normal. 



As in periodic deviations. (See 
table preceding. ) 



As in periodic deviations. (See 
table preceding. ) 

As in periodic deviations. (See 
table preceding. ) 



Esophoria and the Development of Convergent Squint. — 1. De- 
velopment of Squint from a Convergence-excess. — A convergence-excess, 

as shown in the table, has the following characters: 

Esophoria or evidences of inward deviation by all tests, pronounced 
for near, slight, or absent for distance; convergence near-point and 
prism-convergence normal; prism-divergence normal or nearly so; 
inward and outward excursions of the eyes normal, deviation not in- 
creasing to either right or left. 

Convergence-excess is nearly always accommodative, being due to 
the excessive effort which the patient makes to overcome hyperopia or 
astigmatism. An effort of accommodation regularly implies a cor- 



THE EXTKA-OCULAK MUSCLES 253 

responding effort of convergence, so that as he strains one he also strains 
the other. 

A similar accommodative convergence-excess may be produced 
temporarily by the instillation of atropine or homatropine. The patient 
in this case tries extremely hard to use his failing accommodation, and 
makes the effort to do so even when his accommodation is paralyzed. 
To this effort his accommodation cannot respond, but his convergence 
does so, producing a temporary marked deviation. 

A similar variety of convergence-excess, due to the attempt to 
employ a subnormal accommodative power, occurs when the eyes are 
used too soon after debilitating diseases, as measles, etc. 

A convergence-excess may also be caused by an attempt to see 
clearly in conditions of difficult vision due to opacities of the media, 
low degrees of astigmatism, or even a slight amount of myopia. 

In some cases of accommodative convergence-excess the peculiar 
conditions present cause an esophoria that, contrary to the general 
rule, is more marked for distance than for near. Thus, an adult 
with a high amount of hyperopia may succeed in overcoming it for 
distance, but in overcoming it may use so much accommodation as to 
produce also a considerable degree of convergence, while for near- 
points he may be utterly unable to maintain the necessary accommo- 
dation, and hence neither accommodates nor converges. 

Occasionally a convergence-excess is met with in which no ac- 
commodative element is present. 

The use of prisms, base out, especially for constant wear, often 
produce a convergence-excess or decidedly aggravates one already 
present. 

Cases of true spasm of convergence (spastic contraction of the 
interni producing a marked convergent squint) are sometimes seen, 
especially in hysteria (Hitzig cited by Graef e ; Fuchs). 

A convergence-excess when moderate may disappear. This, for in- 
stance, happens in adults who, because of their advancing years, no 
longer exert enough accommodation to overcome their hyperopia, and 
hence give up the effort and with it the excessive convergence. 

In other cases a convergence-excess, especially when it occurs in 
childhood and when of high degree, tends to increase and ultimately 
develops into a convergent squint. The steps by which this occurs are 
as follows: 

(1) A child with a good deal of hyperopia or astigmatism, as soon 
as he begins to make much use of the eyes for near work, develops, as 
a result of undue accommodation, a very varying, evidently more or 



254 THE EYE AKD NERVOUS SYSTEM 

less spasmodic esophoria, which is marked for near when his con- 
vergence is called into play, but which is little or not at all marked for 
distance. The result is either asthenopia, dne to the troublesome 
effort to overcome the deviation, or else a confusing condition of alter- 
nating diplopia and single vision. Usually at this period there is still 
binocular fixation even for near, although every now and then when 
the convergence is particularly called upon (for instance, when a pencil 
at which the eyes are directed is carried quickly toward the nose), 
one of the eyes may turn suddenly and sharply in, producing a momen- 
tary spasmodic squint. 

(2) Little by little the deviation increases until binocular fixation 
for near becomes impossible, and the periodic esophoria is transformed 
into a periodic convergent squint. This at first is intermittent and 
occasional, afterwards more and more constant. It will now be found 
that there is more or less esophoria for distance also, although always 
less than for near. There is still, however, binocular fixation for 
distance. 

Careful tests at this stage will show in nearly every case that the 
squint is associated with diplopia. This diplopia which presents sud- 
den spontaneous variations in amount from one moment to another, 
and which also varies with the distance of the test-object from the eyes, 
is evidently a source of not a little trouble and confusion. 

(3) After this condition has lasted for a time, perhaps for some 
years, the patient gives up binocular fixation for distance, so that he 
now has a convergent squint for both distance and near {continuous 
squint). The deviation as measured by the screen, however, is still 
much more marked for near than for distance. Diplopia can still 
usually be elicited by the tests. The condition is still mainly one of 
convergence-excess. 

(4) Later still, the amount of deviation for distance begins to 
increase until ultimately the squint for distance equals that for near- 
points, and both are absolutely greater than they were. This change is 
due to the superadding of a divergence-insufficiency to the primitive 
convergence-excess; the conditions now, in fact, are those shown in 
the table as obtaining for a convergence-excess and divergence-insuffi- 
ciency combined. The rotations of the eyes are still normal in amount, 
indicating that the muscles themselves are as yet unaffected. 

At this stage, diplopia, if it can be elicited at all, no longer ob- 
trudes itself upon the patient's notice or causes confusion. In many 
cases it cannot be elicited by any tests, suppression of one of the 
images having occurred (page 187). 



THE EXTRA-OCULAR MUSCLES 255 

(5) Later, secondary muscular changes (contracture of the interims, 
stretching and weakening of the externus) take place in the squinting 
eye, producing an absolute reduction in the rotation outward and ex- 
cess of rotation inward. When this occurs we can speak of the squint 
as being in a sense monocular, but until then it must be regarded as the 
result of a strictly binocular process. 

2. Development of Convergent Squint from a Divergence-insufficiency. — 
A divergence-insufficiency, as shown in the table, has the following char- 
acters : 

Marked esophoria and very low diverging power, or, often indeed, 
insuperable or varying homonymous diplopia for distance ; normal or 
nearly normal relations for near ; no impairment of outward nor excess 
of inward rotations of the eyes. 

As a primary affection it is rather rare, although I have seen several 
well-marked instances. Often, especially when slight, it seems to re- 
main unchanged indefinitely ; in other cases it may increase, producing a 
convergent squint. This it does apparently first by the production of an 
increase of the esophoria for distance, then by the superadding of a con- 
vergence-excess, producing an esophoria for near; the result is, first a 
periodic convergent squint (esotropia for distance, esophoria with bin- 
ocular fixation for near) ; later a continuous squint for all ranges. LTntil 
late in their course such cases are distinguished from those starting with 
a primitive convergence-excess by the fact that the deviation for distance 
always exceeds that for near. 

Some cases of convergent squint develop suddenly under the guise 
of a divergence-paralysis. This must be regarded simply as an extreme 
form of a divergence-insufficiency in which the diverging power is not 
simply diminished but abolished. It is characterized by homonymous 
diplopia, which is very great in amount for distance and diminishes 
with mathematical regularity as the test-object is brought toward the 
eyes, and which, instead of growing greater, tends to grow less as the 
eyes are directed to the right or to the left; marked esotropia for dis- 
tance diminishing progressively as the object of vision is carried toward 
the eyes and finally changing to orthophoria at some near point; no 
restriction in the movement of either externus or excess of action of 
either internus, so that the excursions of the eyes are normal. Vertigo 
is often marked and there may be false projection. 

Divergence-paralysis usually develops suddenly. In some seven of 
the reported cases, including two of my own, organic cerebral lesion 
appeared to cause the difficulty, there being a history of syphilis, tabes, 
multiple sclerosis, lead-poisoning, or the evidences of cerebral tumor. 



256 THE EYE AND NEKVOITS SYSTEM 

Another case appeared to be due to concussion of the brain, and still 
another to an antecedent pneumonia and pleurisy. The condition in two 
of UhthofFs cases Avas preceded by bilateral abducens paralysis, and 
one of Bielschowsky's appeared to go into a unilateral paresis of the 
abducens. In many cases the affection occurs without obvious cause. 
Its duration is uncertain, some cases lasting at least four or Rye years, 
others recovering in a year or so. 

Exophoria and the Development of Divergent Squint. — 1. Develop- 
ment of Squint from a Convergence-insufficiency. — Most cases of divergent 
squint develop from a convergence-insufficiency. This has the following 
character : 

Exophoria, marked for near, slight for distance and not increasing 
to either right or left ; convergence near-point remote, being seven centi- 
metres or more from the root of the nose ; prism-convergence subnormal 
and acquired with difficulty; prism-divergence about normal; rotation 
of the eyes inward and outward, normal. 

In many cases the convergence-insufficiency is accommodative, that 
is, it occurs in those who do not require to use their accommodation in 
the normal way. The accommodation and convergence being intimately 
associated, the disuse of one means the disuse of the other. This disuse 
of the convergence often shows itself at first simply by the presence 
of a large amount of exophoria for near without any recession of the 
convergence near-point, but later it develops into a true .convergence- 
insufficiency. 

Accommodative convergence-insufficiency occurs in myopes who are 
not using concave glasses, and may also occur (from sudden relaxation 
of the accommodation) in hyperopes and presbyopes when putting on 
convex glasses for the first time. A somewhat similar relaxation of 
the convergence is found in those who for any reason (presbyopia, 
marked hyperopia, anisometropia, etc.) do not see well for near; 
and in this case the convergence-insufficiency may be relieved by con- 
vex glasses. 

A non-accommodative convergence-insufficiency, — i.e., one which 
develops without any reference to a refractive error, — occurs quite often 
in hysteria, traumatic neuroses, neurasthenia, anaemia, and debilitated' 
conditions, however caused ; also in connection with intranasal disease, 
and not infrequently seems to occur in those who are apparently healthy. 
According to Guillery it may be produced by poisons (alcohol, ether, 
chloroform, and chloral) . 

A convergence-insufficiency, especially if slight, may remain un- 
changed indefinitely ; in other cases, particularly if of large amount, it 



THE EXTKA-OCULAK MUSCLES 257 

develops into a divergent squint. The successive stages of the process 
will then be as follows : 

(1) Marked exophoria for near with at first a normal near-point of 
convergence; orthophoria for distance and normal diverging power 
(periodic exophoria). 

(2) Beginning divergent squint for near with recession of the con- 
vergence near-point; beginning exophoria for distance with increase 
in the prism-divergence (periodic divergent squint). This squint, at 
first intermittent, is afterwards constant. 

(3) Marked increase of the exophoria for distance with gradual 
transformation into squint (consecutive divergence-excess) converting 
the periodic into a continuous squint. 

(4) Muscular changes (contracture of the externus, stretching and 
weakening of the internus) producing absolute impairment of rotation 
inward and excess of rotation outward. 

In some cases a periodic divergent squint develops suddenly from a 
convergence-paralysis. This is characterized by crossed diplopia and ex- 
otropia, which both increase with mathematical regularity as the test- 
object is carried towards the eye. The deviation and the diplopia do not 
increase either to the right or to the left. Movements of each eye out- 
ward and inward are normal. 

A peculiar symptom of convergence-paralysis is the absence of the 
convergence-reaction of the pupil. If, as is usually the case, the light 
reaction is present, the appearance presented is the converse of the 
Argyll-Robertson pupil. Accommodation is sometimes diminished or 
absent, sometimes normal. 

Convergence-paralysis often develops suddenly and is frequently 
associated with very marked vertigo (Parinaud) and with more or less 
false projection (M. Sachs). It is not very infrequent, occurring in 
tabes, multiple sclerosis (in 3 per cent, of the cases), and probably as a 
result of other organic nervous diseases. It is sometimes associated 
with paralysis of divergence or with paralysis of up and down move- 
ments (Parinaud). I have seen a marked instance of the former com- 
bination in a woman who, at a distance of twenty inches, had ortho- 
phoria and, an inch or two further out, homonymous diplopia, increas- 
ing pari passu as the object of vision was carried off, while within 
twenty inches she had crossed diplopia, increasing fast as the object 
was carried towards the eyes. 

A similar absence of divergence and convergence power, constituting 
what A. Graefe calls diminution in the amplitude of fusion, is found 
in multiple sclerosis (Uhthoff) and paralysis of the ocular muscles due 
17 



258 THE EYE AND NEKVOUS SYSTEM 

to central disease (Soelberg- Wells). This condition is marked by the 
fact that the patient cannot overcome any amount of diplopia, however 
minute, produced by prisms. Such inability to overcome prisms, how- 
ever, may also be due to sudden failure of the fusion faculty (p. 185). 

Convergence-paralysis may get well spontaneously or remain in- 
definitely. 

The anatomical site of the lesion is probably in the fibres connecting 
the non-decussating fasciculi of the two internal recti nuclei with each 
other, or connecting these fasciculi with the cortex (Bernheimer) ; or 
it may be cortical. 

2. Divergent Squint developing from a Divergence-excess. — The charac- 
ters of a divergence-excess are as follows : 

Exophoria by all tests, pronounced for distance, slight or absent 
for near ; near-point of convergence and prism-convergence normal ; 
prism-divergence excessive, being over 9° ; movements of eyes inwards 
and outwards normal ; deviation not increasing to either right or left. 

This condition often remains unchanged for a long period, even for 
years. In not a few instances, however, it develops into a divergent 
squint. The steps in the process in this case are as follows : 

( 1 ) Marked exophoria for distance with excessive prism-divergence ; 
exophoria for near, little or none, and near-point of convergence nor- 
mal (periodic exophoria). 

(2) Beginning divergent squint for distance with crossed diplopia 
and still further increase in the prism-divergence ; beginning exophoria 
for near, but with convergence near-point still normal (periodic diver- 
gent squint). This squint is at first intermittent, afterwards constant. 

(3) Marked increase of the exophoria for near and gradual reces- 
sion of the convergence near-point (consecutive convergence-insuffi- 
ciency), producing a squint for near, which, however, is less than for 
distance). 

(4) Marked divergent squint which is of about the same amount 
for both distance and near (conversion of the periodic into a continuous 
squint by the superadding of a convergence-insufficiency to the primi- 
tive divergence-excess) . Diplopia still often present, but now usually 
negligible by the patient. The rotations of the eye inward and outward 
are still about normal. The patient may still make strong converging 
efforts, so that though he has no longer an absolute he has still a rela- 
tive convergence near-point. 

(5) Muscular changes (weakening of the interni ; contracture of 
the externi) causing impairment of rotation inwards and excess of rota- 
tion outwards. 



THE EXTRA-OCULAR MUSCLES 259 

Rationale of the Development of Squint. — How and when Squint 
develops. — A squint in most cases apparently arises from an instinctive 
endeavor to avoid diplopia and confusing images. It seems quite cer- 
tain that in their inception the different periodic deviations are accom- 
panied by diplopia. If the deviation is slight the patient overcomes 
the double vision with ease; if it is great, with more and more diffi- 
culty. In the latter case ultimately he gives up the effort to overcome 
the diplopia and then sees double all the time. 

Xow it becomes apparent when we study these cases that the more 
nearly continuous (non-periodic) the deviation is the easier it is for 
the patient to ignore the diplopia to which it gives rise, and the less 
pronounced, consequently, are the subjective disturbances which are 
dependent on this diplopia. 

Again, it is almost always the case that a large deviation causes 
much less subjective trouble than does a small one : first, because the 
image projected by the deviating eye is so faint and so far from the 
other that the patient can readily ignore it; second, because the devia- 
tion being so large that the patient cannot possibly overcome it, he does 
not even make the attempt to do so, and thus escapes the reflex disturb- 
ance that such an attempt often produces. Accordingly, it is for the 
patient's interest that if he has a periodic deviation it shall be trans- 
formed into one that is as nearly continuous as possible ; and, second, 
that if it is small it shall increase in amount until the diplopia is readily 
negligible. 

As we have just seen, both kinds of change occur regularly in the 
development of heterophoria, and particularly in the evolution of con- 
vergent and divergent squint. 

The processes by which these changes take place are not located in 
the muscles themselves, for until very late in the development of most 
cases of squint the rotations of the eyes are normal, indicating that the 
power of the muscles per se is neither impaired nor excessive. The 
changes that take place are doubtless central, following, as Hoffmann 
pointed out, the law promulgated by Sherrington, that excessive stimu- 
lation of the centre for one movement produces inhibition of the centre 
for the opposing movement, and vice versa. In other words, in cases of 
convergence-excess the centre for convergent movements being subjected 
to continuous overstimulation, becomes more and more active, producing 
a continually greater excess of convergence action and at the same time 
causing greater and greater inhibition of the centre for the divergent 
movements. The consequence is that superadded to the convergence- 
excess we have ultimately a progressive divergence-insufficiency. It 



260 THE EYE AND NERVOUS SYSTEM 

is only late in the course of the condition that the persistent stimulation 
of the internus, coupled with the maintenance of one eye in the forced 
inward position, produces hypertrophy or contracture of this muscle 
and stretching or atrophy of its opponent. 

A small proportion of cases of squint do, however, develop out of a 
true muscular defect. Thus, some cases of convergent squint, both in 
childhood and in adult life, develop out of a primitive paralysis of the 
abducens which has been converted into a comitant deviation by con- 
secutive overaction and contracture of the internus (page 243). Fur- 
thermore, a convergent squint due to a convergence-excess may be 
aggravated by the presence of a primitive weakness of the externi or 
overdevelopment of the interni. Again, a divergent squint may be 
produced by developmental changes in the orbit causing a relative pre- 
ponderance of the externi (page 221), or by weakness of the internus 
due to operation. Such factors, however, play a very subordinate part 
in the production of squint. 

Conditions favoring the Development of Squint; Period at which it de- 
velops. — The development of a convergent squint out of a heterophoria 
in the way just outlined is much more apt to occur if there is any con-, 
dition such as anisometropia, opacities of the media, fundus disease, 
congenital anomalies, etc., that renders the sight of one eye much worse 
than that of the other. In such a case the image of the worse eye often 
confuses that of its fellow instead of reinforcing it, and hence the patient 
really sees better when he allows the worse eye to deviate. In many 
cases it is apparent that he actually forces it to deviate so as to get the 
disturbing image out of the way. This is particularly the case in diver- 
gent squint. 

A factor which may be of considerable importance and which, in- 
deed, by many is regarded as the predominating element in the pro- 
duction of squint is the lack of development of the fusion-faculty (page 
185). The effect of this would be that the patient would have np ten- 
dency to blend the two retinal images, and hence would have no spe- 
cial motive for maintaining binocular fixation. Consequently, if there 
was a deviation of the eyes, he would not try to overcome it, but would 
allow one of them to squint. 

Ill-development of the fusion-faculty and the squint that it produces 
may be hereditary. Some, indeed, go so far as to regard heredity as 
the main factor in the genesis of squint. This view, I think, is not 
substantiated by the facts. 

A convergent squint usually develops in early childhood. Once 
developed, it increases to a certain point, then generally remains un- 



THE EXTKA-OCULAR MUSCLES 



261 



changed through life. In not a few cases, however, it diminishes in 
later childhood, and may even disappear altogether or be converted into 
a divergent squint. These changes are probably due to developmental 
processes (divergence of the orbits during the growing period), which 
tend to render the power of the externi greater and that of the interni 
less (see Weiss, page 221). 

Squint develops very often in congenital spastic paralysis (Little's 
disease). 

Divergent squint generally occurs later in life and is more often 
accompanied by persistent diplopia than is convergent squint. It some- 
times develops after a tenotomy done for convergent squint, and this 
may occur even years after the operation. This secondary exotropia 




is traceable sometimes to an insufficiency of the interims produced by 
the tenotomy ; more often it is not attributable to the latter, being due 
either to the developmental changes (divergence of the orbits) above 
mentioned, or to the supervention of a true divergence-excess. 

Convergent squint occurs about three times as often as divergent 
squint.* In anisometropia, however, divergent squint is the more fre- 
quent form. 

Squint a Bilateral Affection.' — In squint the patient appears simply to 
fix with one eye and to turn the other inwards or outwards. In reality, 
however, both eyes are turned. Thus, as shown in Fig. 15, when a 

* The disproportion is greater than this if we judge from most hospital sta- 
tistics, but this probably arises from the fact that slight degrees of divergent squint 
are frequently overlooked. 



262 THE EYE AND NERVOUS SYSTEM 

patient with convergent squint is told to look at the object A, both eyes 
tend to converge equally, so that they really look at some nearer point, 
like B ; then, in order to get the image of A on the fovea of at least 
one eye, the patient turns both eyes to one side — say to the right — 
through the equal angles BKA and BLD. The right eye, R, is now 
directed towards A or fixes it, the left eye squints in by the angle 
ALD=2 X BLD. Thus, here really the squint is bilateral, each 
eye actually turning in by an equal amount, yet apparently the 
left eye alone squints, because the patient happens to use the right for 
fixation. 

Which eye the patient actually uses to fix with depends usually upon 
the vision. If he sees much better with one eye than with the other he 
will fix with the better and squint with the worse eye; the squint will 
then be unilateral. If the two eyes are about alike in vision and refract- 
ing power, we often find an alternating squint, the patient using either 
eye indifferently for fixation and squinting with the other. In some 
cases, particularly in anisometropia, the patient may fix with one eye 
for distance and with the other for near. 

Hyperphoria and Vertical Squint. — Hyperphoria in a great many 
instances is caused by paresis, or at least insufficiency of one of the 
elevator or depressor muscles of the eye. In this case the deviation and 
its evidences (vertical diplopia, etc.) increase and decrease character- 
istically in looking in some particular direction of the gaze (page 215). 
Hyperphoria of high degree and real vertical squint (hypertropia and 
hypotropia) are almost always of this origin, being, therefore, non- 
comitant deviations. 

In some cases, even when thus originating, a hyperphoria or vertical 
squint may become nearly comitant by virtue of consecutive contracture 
of the opponents of the paretic muscles (page 243). 

In a few instances even a very high degree of hyperphoria or a true 
vertical squint may be truly comitant from the start, — i.e., we can find 
no evidence of insufficiency of any elevator or depressor muscle. Hyper- 
phorias of low degree are usually comitant. 

Some cases of hyperphoria seem to be spasmodic in origin, changing 
rapidly and lasting only a short time. Sometimes a spasmodic hyper- 
phoria of this sort seems to be due in some way to accommodative action, 
since it disappears apparently as the result of the correction of a re- 
fractive error. In most cases hyperphoria once developed lasts indefi- 
nitely. It does not, as a rule, tend to increase, except in the form due 
to marked paralysis of an elevator or depressor where the deviation is 
often exaggerated as time goes on, either by secondary contractures of 



THE EXTKA-OCULAK MUSCLES 263 

the opposing muscles or by a secondary deviation in the good eye due 
to fixation with the one that is paretic (page 215). 

Symptoms of Heterophoria and Squint. — These are — 

1. Diplopia. — This, especially when slight so that the double images 
are close enough together to confuse each other, produces blurring of 
sight and sometimes vertigo. 

2. Reflex Symptoms induced by the Effort made to correct the Devia- 
tion. — These are asthenopia, pain in the eyes, conjunctival irritation, 
headache, which may be supraorbital, orbital, temporal, or occipital, 
and other pains situated in various parts of the body. Occasionally, 
also, we find spasm in the facial muscles, and sometimes other forms 
of spasm; not infrequently also digestive disturbances, nausea, and 
interference with the general nutrition. 

Reflex symptoms are especially pronounced when the deviation is 
so slight that the patient can overcome it, and consequently does so, 
although with effort. They are particularly marked when the deviation 
is inconstant and varies under different conditions, because in this case 
the patient cannot as readily allow for the changing position of the 
eyes as he can when the deviation is the same all the time. Conse- 
quently, reflex disturbances are greater in heterophoria than they are 
in squint and greater in a periodic squint than they are in one which is 
continuous. Indeed, as we have seen, this very fact seems to constitute 
the reason why a periodic is regularly converted into a continuous 
squint. 

Both the tendency to diplopia and the reflex disturbances caused 
by muscular anomalies increase in proportion to the difficulty that the 
patient meets with in maintaining binocular fixation. They are hence 
greater under conditions demanding very accurate and very sustained 
fixation, — greater, for instance, when the patient is doing continuous 
fine work or when he is looking steadily at brightly illuminated or 
sharply defined objects, particularly when such objects are in motion. 
Hence, many persons with moderate muscular anomalies always suffer 
from headache and fatigue (panorama headache, panorama asthenopia) 
after shopping or going to the theatre, or after seeing a ball-match or 
a street-parade. 

The most marked and disagreeable symptoms are found in hyper- 
phoria and in divergence-insufficiency. In these conditions the symp- 
toms are usually most troublesome in distant vision. 

In convergence-insufficiency the commonest symptoms are asthen- 
opia, headache, and conjunctival irritation, and these are developed 
generally by near work. They are much more apt to occur in the non- 



264 THE EYE AND NEKVOUS SYSTEM 

accommodative than in the accommodative form of convergence-insuffi- 
ciency. 

Treatment of Heterophoria and Squint. — 1. Correction of Refrac- 
tion. — This is a very important element in all of these anomalies and 
may alone suffice to effect a cure, particularly when the deviation is 
still of the periodic type. It is of special importance and efficacy in 
convergence-excess, particularly convergence-excess which is developing 
into squint. In this condition it is necessary to correct the whole of 
the hyperopia and astigmatism present. Similarly, in convergence- 
insufficiency accompanying myopia, it is of great importance to make 
the patient wear a glass fully correcting his error and for near as well 
as for distance. 

In cases in which the deviation is no longer periodic, — i.e., in which, 
in addition to a convergence anomaly, there is superadded an anomaly 
of divergence, — correction of the refraction is less apt to afford a cure, 
and in the case of a continuous squint very rarely does more than 
diminish the amount of the deviation. In any case, the effect of cor- 
rection of refraction is rarely an immediate one; it usually takes some 
months — perhaps a year or two — for the full effect of the glasses to be 
secured. 

2. General treatment must he employed in neurasthenia and hysteria 
and in conditions of debility from whatever cause. This will often 
relieve the symptoms without itself producing any material change in 
the muscular condition of the eyes. 

3. Treatment of Associated Conditions. — The treatment of other affec- 
tions, particularly intranasal disease, which may possibly be sources 
of reflex trouble, should never be neglected. 

4. Exercises in moving the Eyes. — Exercises of the eyes in converging 
on a pencil carried towards the nose or in overcoming prisms, base out, 
are useful in convergence-insufficiency and occasionally in divergence- 
excess. 

5. Orthoptic Exercises. — With a squint that develops in early life 
much can be done by educating the fusion faculty. This can be done 
with the stereoscope, or, better still, with Worth's amblyoscope (Fig. 
8). Both of -these instruments, by compelling the eyes to act together, 
train the fusion-faculty. Both are at first adjusted for the patient's 
angle of squint, then by successive adjustments the patient is gradually 
taught to look into the instruments with the eyes more and more nearly 
straight and yet maintaining fusion. In this way the angle of squint is 
steadily diminished, and there is finally orthophoria. 

Another orthoptic exercise is bar-reading. This consists in reading 



THE EXTKA-OCULAK MUSCLES 265 

with a bar held between the eyes and the print. Unless the patient is 
using both eyes at once for seeing, the bar will hide some of the letters. 
The use of the bar is an exercise in binocular vision. 

The squinting eye can also be exercised either by bandaging the 
good eye, or, better still, by keeping the latter for some weeks under 
atropine. 

6. Prisms for Wear. — Deviations of low degree may be corrected by 
prisms worn constantly, either alone or combined with the glass cor- 
recting the refraction. This is most serviceable in hyperphoria. In 
esophoria and exophoria, prisms constantly worn often tend to increase 
the deviation, and their use is not generally advisable. Moreover,, it is 
not usually possible for a patient to wear prisms of 3°, or at most 4°> 
before one eye, so that the total amount of deviation that we can correct 
by this means would not be more than 7° at most. 

7. Operation. — When everything else has been tried and has failed, 
an operation is indicated. 

With an esophoria or convergent squint, tenotomy of one or, better, 
of both internal recti is done, provided the condition is mainly one of 
convergence-excess. When the condition is one of divergence-insuffi- 
ciency, the better plan is advancement of one or both externi. 

Similarly, in exophoria or divergent squint caused by divergence- 
excess, tenotomy of one or, better, of both external recti, is indicated. 
Cases of convergence-insufficiency, on the other hand, should be treated 
by advancement of the interni. In marked divergent strabismus tenot- 
omy of one or both externi, combined with advancement of the interni, 
usually has to be done. 

In hyperphoria, if non-comitant, we proceed according to the rules 
laid down for operative interference in paretic deviations (page 215). 
In a comitant hyperphoria the best operation is a tenotomy of the 
superior rectus, which should never be pushed so far as to render the 
muscle paretic nor so far as to produce even a moderate overcorrection. 

Section V. 

DERANGEMENTS OF THE ASSOCIATED PARALLEL 
MOVEMENTS OF THE EYES. 

The derangements of the associated parallel movements of the eyes 
comprise — 

A. Paralysis and spasm of the parallel movements (conjugate 
paralysis, conjugate spasm). 

B. Tremor and ataxia of parallel movements (nystagmus, pseudo- 
nystagmus). 



266 THE EYE AND NEKVOUS SYSTEM 

Conjugate Paralysis and Spasm. 

Nature of these Anomalies. — We not infrequently meet with cases 
in which the ability of the eyes to perform some parallel movement is 
impaired or abolished (conjugate paralysis). Thus, both eyes may move 
from left to right as far as the middle line, and then stop short, refusing 
to go any farther (paralysis of dextroversion). In this case commonly 
the two eyes and sometimes also the head are deviated in the opposite 
direction,* — i.e., show a conjugate deviation to the left. This conjugate 
deviation in a paralysis of the right-rotators is caused by an overaction 
of the antagonists, or left-rotators. Such overaction is probably central 
in origin, since Sherrington's experiments seem to indicate that abolition 
of function in the nerve-centre presiding over any movement is regularly 
associated with exaltation of function in the centre that presides over 
the opposing movement. 

In some cases conjugate deviation is due not to a conjugate paralysis, 
but to a conjugate spasm. Eor example, we may find both eyes deviated 
to the left, not because the right-rotators are paralyzed, but because the 
left-rotators are in a state of primary spastic contraction. Such a con- 
jugate spasm results from an irritative lesion of the centre for left- 
rotation, just as a conjugate paralysis results from a destructive or 
inhibiting lesion of the centre for right rotation. It is distinguished 
from paralysis by the fact that, although strongly deviated in one direc- 
tion, the eyes can usually be moved more or less freely in the direction 
opposite. 

Symptoms. — Since the deviation is alike in the two eyes, conjugate 
paralysis and spasm do not cause diplopia. They may, however, cause 
vertigo and false projection (A. Graefe; M. Sachs). In a conjugate 
deviation to the left, due to paralysis, the patient would overshoot the 
mark when trying to touch an object situated on his right, while if the 
deviation were due to spasm he would undershoot the mark when trying 
to touch an object situated on his left. 

Very frequently in cases of conjugate deviation any symptoms that 
may be occasioned by the eye trouble are masked by the grave general 
symptoms due to the causal disease. 

Course. — Conjugate paralysis occurs in all degrees of intensity, 

being sometimes very slight, sometimes complete. It usually develops 

suddenly, and is often transient. The conjugate deviation which the 

paralysis produces often disappears long before the paralysis itself. 

This is especially true of cerebral disease. Here the conjugate devia- 

* In rare cases the head may be deviated in one direction and the eyes in the 
direction opposite {alternating conjugate deviation of Bresler). 



CONJUGATE PAKALYSIS AND SPASM 267 

tion, which is quite pronounced during the period of coma, often dis- 
appears within a few hours, and rarely lasts more than three or four 
weeks, although when it does go it generally still leaves a partial con- 
jugate paralysis behind. 

Non-paralytic Restriction of Conjugate Movements; Diagnosis 
from True Paralysis. — A conjugate paralysis must be differentiated 
from the condition in which the patient can move the eyes in the given 
direction, but does not move them, either from wish to deceive or from 
fear of pain, or because he really does not know how to make the effort. 
In some cases, according to Gunn, the patient, when told to look to one 
side, or up, or down, seems unable to initiate the movement of his oavii 
will, although he can follow a slowly -moving object that is carried in the 
given direction. In other cases he cannot even do this, and yet the power 
of movement, although apparently never exerted, is really there, as we can 
prove by Graefe's test. This test is best performed by making the patient 
look at a distant object, and then placing a prism of 10° or 20° before 
each eye, with the apex directed in the way in which we wish the eyes 
to move (e.g., to the right in paralysis of dextroversion, etc.). If the 
patient really has a conjugate paralysis, his eyes will move neither 
when the prisms are put before them nor when they are taken away. 
If, however, he can move the eyes, we shall see each make a dis- 
tinct jump in the direction of the prism-apex when the prisms are 
put on, and a distinct jump in the opposite direction when they are 
taken off. 

More or less allied to conjugate paralysis is the curious condition 
of inertia of the ocular movements described by Gowers as occurring 
in a case of progressive muscular atrophy. The patient when gazing 
.at one object was told to look at another situated 45° to the right or 
left. He turned his head so as to front the second object, but kept his 
eyes still fixed on the first, and only after a time, and slowly then, trans- 
ferred his gaze from this object to the one he was told to look at. 

Varieties of Conjugate Paralysis and Spasm. — 1. Paralysis of Side- 
to-Side Movements (Lateri version). — Paralysis of a side-to-side move- 
ment (e.g., of the conjugate movement to the right) occurs under the 
following forms : 

(A) Xeither eye, when attempting to follow an object that is 
moving to the right, can move much or at all beyond the middle line 
(paralysis of dextroversion, right and left). But the left eye can 
move to the right when converging on an object which is brought in 
towards the eyes in the median plane (retention of convergence). The 
condition here is, then, a pure paralysis of lateriversion, and the 



268 THE EYE AND NEKVOUS SYSTEM 

appearances it presents are, it will be seen, the precise opposite of those 
that obtain in a paralysis of convergence (page 257). 

(B) Neither eye can follow an object that is carried to the right, 
provided both eyes are open, bnt the left eye can follow the object to 
the right if the right eye is covered. This is really the same case as 
A, the movement inwards of the left eye when the right eye is covered 
being simply a movement of convergence ( Jeffries, Mobius). 

Two unusual cases of this type have been reported in which there 
was paralysis of the movements of both eyes to the right, provided 
both eyes were open, but in which the right as well as the left eye could 
move to the right when separately tested. In one case (Samelsohn's) 
each eye could move to the right if the eye itself was screened ; in the 
other case (that of Stevens of Denver) each eye could move to the 
right if the other eye was screened. In both cases the individual 
movement of the right eye to the right was probably a movement of 
active divergence (page 196) just as the movement of the left eye to the 
right was one of active convergence. 

(C) The right eye can follow an object that is moving to the right, 
but the left cannot. The left eye, however, as in type A, can move 
to the right in performing convergence. This condition, which may 
be called conjugate hemiparalysis (paralysis of left dextroversion), is 
distinguished from a paralysis of the left internus by the fact that in 
the latter the left eye could move inwards neither when converging nor 
when attempting to make a conjugate movement to the left. 

(D) Neither eye can follow an object that is carried to the right, 
and the left eye, moreover, cannot move to the right when attempting 
to converge (combined paralysis of lateriversion and convergence). 

Paralysis of lateriversion is sometimes bilateral, — i.e., both eyes 
fail to follow an object that is carried either to the right or to the left, 
although it may be that both can still converge. 

2. Spasm of lateriversion occurs under the following forms : 

(A) Both eyes are deviated spastically to one side, but can be 
moved freely in all directions (pure spasm of lateriversion). 

(B) Both eyes are deviated spastically to one side, and cannot be 
moved to the side opposite (excessive spasm of one side-to-side move- 
ment, or spasm of one side-to-side movement combined with paralysis 
of the movement in the opposite direction). 

3. Paralysis of Up-and-Down Movements. — Paralysis of the move- 
ment of both eyes upwards (sursum version) or downwards (deorsum- 
version) is uncommon. Cases are reported by Nieden, Parinaud, 
Gowers, Sauvineau, A. Graefe, M. Sachs, Snell, KornilofT, Posey, etc. 



CONJUGATE PARALYSIS AND SPASM 269 

It is probable that some of the cases reported are spurious, the 
power of movement being present but never put forth. This was so 
in one of my own cases, in which repeated examinations extending over 
several years always showed complete inability of the eyes to go up- 
wards above the horizontal plane, and yet Graefe's test (page 267) 
proved that they could really turn up at least 30 °, and subsequently 
the patient regained the power of spontaneous movement. 

Paralysis of sursumversion and deorsumversion sometimes occur 
together, the eyes being unable to move either up or down (Lang and 
Pitzgerald, Wernicke, Graefe, UhthofT), and both conditions are often 
associated with paralysis of convergence (in one of my cases apparently 
with paresis of divergence). 

4. Spasm of up or down conjugate movements may occur, as in the 




Fig. 16.— Paralysis of sursumversion and of convergence. Case of Dr. Posey. 

case reported by Frost, in which both eyes were directed down and 
to the right and remained rigidly fixed in this position. Or, as in a case 
reported by me, there may be an intermittent clonic spasm of the oblique 
movements, so that the eyes at intervals darted obliquely far up and 
to the right and returned more slowly to the primary position. Such 
a condition occupies an intermediate place between true spasm and 
nystagmus (Compare Chapter IX). 

Site of Lesion in Conjugate Paralysis and Spasm. — Paralysis and 
spasm of lateriversion may be produced by lesions in the cerebrum, cere- 
bellum, or pons. 

Cerebral lesions, especially those of sudden onset and associated with 
apoplectoid symptoms (coma, hemiplegia), often cause paralysis of 
lateriversion. A lesion of the right hemisphere produces paralysis of 



270 THE EYE AND NEKVOITS SYSTEM 

movements to the left, and consequently a conjugate deviation to the 
right. Or as Prevost puts it, the patient turns his eyes towards the 
lesion. 

The lesion may be situated at various portions of the cortex, or 
anywhere in the subcortical association paths, or in the tracts connect- 
ing the cortex with the third and sixth nerve nuclei. 

Sometimes a cerebral lesion produces a spasm of lateriversion. In 
this case, if situated on the right side, it will cause a spastic deviation 
to the left, — i.e., the patient consequently turns his eyes away from the 
lesion. 

According to Bernheimer, a spastic conjugate deviation, if due to 
cerebral disease, occurs only in cortical lesions (e.g., in meningitis of 
the convexity of the brain). 

Conjugate deviations due to cerebral disease are usually most pro- 
nounced during the period of coma, and, after this has passed off, gener- 
ally disappear pretty soon — within a few days or at most a few weeks, 
sometimes within a few hours. Another distinguishing point is that 
in a paralysis of right or left movements of cerebral origin the con- 
vergence is always retained (Bernheimer), so that the picture presented 
is that of a pure paralysis of lateriversion (type A, page 267). 

Conjugate deviation is occasionally produced by abscess of the cere- 
bellum, the eyes being turned sometimes towards the lesion, usually away 
from it (Eversbusch). 

Disease of the pons often causes paralysis of lateriversion, and 
if on the right side of the pons will produce paralysis of right-hand 
movements, and vice versa, — i.e., the eyes look away from the 
lesion. 

Small lesions in the pons involving the abducens nucleus or its 
immediate connections cause paralysis of lateriversion without paralysis 
of convergence (paralysis of types A and B, pages 267, 268). Lesions 
high in the pons involving only the fibres connecting the abducens and 
the oculo-motor nucleus produce a conjugate hemiparalysis (paralysis 
of the opposite internus for parallel movements but not for convergence, 
(type C, page 268). Large lesions in the pons above and below the 
abducens nucleus cause combined paralysis of lateriversion and con- 
vergence (paralysis of type D, page 268). (Jeffries, Schoeler.) 

Very rarely (Schoeler; Eichhorst's case cited by Jeffries) pons 
disease causes a spasm of lateriversion. In this case the eyes looh 
towards the lesion. 

The diagnosis between cerebral and pontine conjugate deviations 
may be made from the following considerations : 



CONJUGATE DEVIATIONS 



271 



CONJUGATE PARALYSIS. 

In both kinds, the eyes cannot be moved in a certain direction, and usually show 
a well-marked deviation in the direction opposite. 

CEREBRAL. 

Deviation transient. 

Abducens paralyzed on same side as 
hemiplegia (eyes look away from par- 
alyzed limbs ) . 



The eye that cannot turn inwards in 
making lateral movements yet can 
always turn inwards in making con- 
vergence movements. 

Always some associated focal symptoms. 

Facial paralysis, if present, on the same 
side as the hemiplegia. 



PONTINE. 

Deviation more apt to be lasting. 

Abducens paralyzed on side opposite to 
hemiplegia (eyes look towards para- 
lyzed limbs). 

Adduction in some cases nil for con- 
vergence, as well as for parallel move- 
ments. 

May be none ( then surely pontine ) . 

Involvement of the facial (usually on 
the side opposite to the body paraly- 
sis) very frequent. Fifth, eighth, and 
twelfth also often involved. 



CONJUGATE SPASM. 
In both, the two eyes are strongly deviated to one side, but can usually be moved 
more or less freely in the direction opposite. 

CEREBRAL. PONTINE. 

Abducens on same side as hemiplegia Abducens on side opposite to hemiplegia 
spastically contracted (eyes look to- spastically contrasted (eyes look away 

wards paralyzed limbs ) . from paralyzed limbs ) . 

The following differential table furthermore will hold good for 
most cases : 

SITE OF LESION IN CONJUGATE DEVIATIONS. 

BOTH EYES ARE DEVIATED TO THE RIGHT.* 



A. Eyes cannot be turned to the left. 

(a) There is L. hemiplegia, with or 

without involvement of cranial 
nerves on L. side. 

Destructive lesion of R. side 
of cerebrum or of L. side of 
cerebellum. 

(b) There is R. hemiplegia and usu- 

ally involvement of fifth, sev- 
enth, and other cranial nerves, 
especially on L. side. 

Destructive lesion of L. side 
of pons. 



B. Eyes usually can be turned to the 
left. 
(a) There is R. hemiplegia. 



Irritative lesion of L. side of 
cerebrum ( cortex only ) , or pos- 
sibly of R. side of cerebellum. 
(b) There is L. hemiplegia with in- 
volvement of fifth, seventh, and 
other cranial nerves, especially 
on R. side. 

Irritative or combined irrita- 
tive and destructive lesion of 
pons. 



Paralysis of upward movement in both eyes seems to be due usually 
to a lesion involving the anterior portions of both third-nerve nuclei 



* For diagnosis of conjugate deviations to the left, substitute R. for L. and 
vice versa. 



272 THE EYE AND NERVOUS SYSTEM 

or the corpora quadrigemina or one occupying the middle line close 
behind the corpora quadrigemina and affecting the gray matter about 
the aqueduct (Henoch, Jeffries, Gowers, Bruce). In a case with the 
paralysis of both up and down movements Wernicke found a lesion of 
the corpus striatum and the optic thalamus. Paralysis of up or down 
movements also occurs occasionally in pons lesions (in seven out of 
one hundred and forty cases collected by Schoeler). Judging also 
from Russell's and from Sherrington's experiments on animals, such 
a paralysis may also possibly be occasioned by a lesion of the anterior 
cerebral cortex. 

Cases simulating a Conjugate Paralysis, but of Basal or Periph- 
eral Origin. — Conditions which closely resemble a conjugate paralysis, 
but which are not really such since they are not due to an affection 
of the centres governing conjugate movement, may be caused by sym- 
metrical basal or peripheral lesions (simulated conjugate paralysis). 

Thus Olbers reported a case of conjugate deviation to the right in 
which autopsy showed that the left internus was altogether absent and 
the right externus nearly so. So also a number of cases of paralysis 
of upward or downward movement are apparently due to symmetrical 
aplasia or mal-insertion of the superior recti and other muscles (Heuck, 
Lawford, Stieren, A. E. Davis). A similar paralysis of upward move- 
ment (Ormerod) or of both upward and downward movement (Uht- 
hoff) may be caused by symmetrical lesions of the two third nerves at 
the base of the brain. In Thomsen's case of symmetrical paralysis of 
upward movement, the lesions were both basal and fascicular. 

Conditions causing Conjugate Paralysis and Spasm. — Conjugate 
paralysis and spasm are often caused by hemorrhage (especially cere- 
bral hemorrhage) and embolism; less frequently by tumors, abscess 
(especially otitic abscess, both cerebral and cerebellar), and meningitis. 
They may also occur in polioencephalitis superior before the stage of 
complete paralysis, indicating, then, that the process has involved the 
gray matter beneath the aqueduct, but not as yet the oculo-motor nucleus 
(Bernheimer). 

Paralysis of conjugate movement, both lateral and vertical, occurs 
quite frequently in multiple sclerosis (in 3 per cent, of the cases, accord- 
ing to Hhthoff). It is also occasionally present in pseudobulbar paraly- 
sis, and, very rarely indeed, in tabes and amyotrophic lateral sclerosis. 

Conjugate paralysis and especially conjugate spasm may be due 
to hysteria, the traumatic neuroses, or neurasthenia. In this case, 
Graefe's test (page 267) may show that the power of movement, although 
apparently absent, is actually present. 



NYSTAGMUS AND PSEUDONYSTAGMUS 273 

Defects in the associated movements of the eyes are sometimes 
congenital and hereditary. Such defects, however, are probably in 
most cases not instances of a true conjugate paralysis, but are simply 
the results of a symmetrical absence of the muscles or of some similar 
anomaly of development (page 272). 

Treatment. — It is rare for a conjugate deviation to give rise of 
itself to any symptoms that require treatment. In cases in which 
the vertigo is annoying and the conjugate deviation persistent, the 
condition may be relieved by a symmetrical tenotomy {e.g., of the two 
right rotators, if there is paralysis of levoversion). (A. Graefe.) 

Tremor of the Associated Parallel Movements. 
Nystagmus and Pseudonystagmus. 
Varieties of Tremor. — We must distinguish three varieties of tre- 
mor of the eye movements : 

A. Searching Movements. — Both eyes make an occasional wide, com- 
paratively slow, sweeping movement from the primary position in 
some direction, and then, either immediately or after a' time, return 
to the primary position. The movements are apparently purposeful, 
just as if the patient were trying to look at an object situated to one 
side of him. They may take place in any direction, vertical, oblique, 
or horizontal. 

B. Pseudonystagmus. — One or both eyes when carried to a point 
near the limit of their excursion in some direction, make a series of 
jerky movements on from this point and back to it again, but in return- 
ing do not re-pass it. These jerks evidently represent an extreme effort 
to keep up the original excursive movement of the eyes, the smooth 
steady pull of the muscle that is carrying the eye along being converted 
into a series of discontinuous, spasmodic tugs. 

The direction of the jerky movements in pseudonystagmus is usually 
the same as that in which the eyes were being carried when the move- 
ments started, — i.e., if the eyes were moving to the right the pseudo- 
nystagmus will make them move jerkily still further to the right and 
then more slowly back again. Sometimes, however, when the eyes are 
moving laterally, the spasmodic attempt to carry them further may 
result, not in a continuation of the lateral movement, but in a jerky 
rolling of the eyes about an anteroposterior axis. 

Pseudonystagmus, although more often bilateral, is not infrequently 
confined to one eye, particularly if that eye has relatively weak muscles. 

C. Nystagmus. — In nystagmus the eyes make a series of very regu- 
lar, short, quick oscillations about a central point. Nystagmus differs 

18 



274 THE EYE AND NEKVOUS SYSTEM 

from pseudonystagmus, first, in that it often occurs when the eyes are 
in the primary position or near it, whereas pseudonystagmus occurs 
only when the eyes are near the end of an excursion in some direction ; 
second, the movements in nystagmus are oscillations to and fro about 
a central point, while in pseudonystagmus they are movements from 
a terminal point and hack to it again ; third, the movements in pseudo- 
nystagmus are jerky and bespeak effort, in true nystagmus are smooth 
and regular, and, except in miners' nystagmus, are not often associated 
with a restriction in the excursive power of the eyes, which is very 
frequently present in pseudonystagmus. 

Number and Extent of Oscillations. — The oscillations in nystagmus 
range from one to four a second and in width vary from one to five 
millimetres. 

In nystagmus the oscillations may be horizontal, vertical, or rotary, 
— the cornea in the last case revolving like a wheel round its centre 
to and fro, through a more or less considerable arc. Combined vertical 
and horizontal nystagmus producing an oblique oscillation is called 
mixed nystagmus; and the same term may with propriety be ap- 
plied to the forms in which a rotary is combined with a lateral or 
a vertical oscillation. Still more complicated are the cases described 
by A. Graefe as occurring in miners' nystagmus, in which, by a com- 
bination of successive horizontal and vertical movements, the centre of 
each cornea moves in a circular or elliptical path {circumduction 
nystagmus). Thompson and Spicer have described a similar circum- 
duction movement in the nystagmus associated with spasmus nutans in 
infants. 

Symmetry of Movements. — The nystagmic movements show a 
marked symmetry. The movements are nearly always bilateral, and 
when bilateral are almost, without exception, equal, simultaneous, and 
parallel in the two eyes, — i.e., both eyes move equally far and at the 
same instant to the right, or both rotate to the right, etc. 

Bilateral nystagmus is usually horizontal, although the rotary form 
is pretty frequent. Mixed nystagmus is less often met with, and ver- 
tical bilateral nystagmus is rare. 

Very rarely indeed bilateral nystagmus is disjunctive, — i.e., the two 
eyes, instead of moving in a parallel path, move towards or away from 
each other. Thus in Graefe's case of circumduction nystagmus the 
eyes both travelled in elliptical paths, but in opposite directions — each 
revolving from the nose towards the temple. I myself saw a case in 
which there was a converging rotary nystagmus, — i.e., both corneas 
made simultaneous wheel-like movements from the temple towards the 



XYSTAGMUS AND PSEUDOXYSTAGMUS 275 

nose. Thompson and Spicer say that infants affected with spasmus 
nutans sometimes show a form of nystagmus in which the eyes alter- 
nately and rhythmically converge and diverge, and the same form has 
been observed in adults as the result of excessive accommodation in 
near work (Eversbnsch). 

Also very rare are the cases of nystagmus in which the motions of 
the eyes are dissociated, — i.e., are quite unlike, so that they do not 
resemble any co-ordinated movement, either parallel or disjunctive. 
Thus, the oscillations may be horizontal in one eye and vertical, rotary, 
or oblique in the other. Such combinations occur (a) in the nystagmus 
of spasmus nutans (Thompson) ; (6) sometimes in a unilateral nystag- 
mus when it assumes the bilateral form (Schapringer, Xeustatter, 
Duane) ; (c) occasionally in ordinary bilateral nystagmus (A. Graefe, 
Frost, Lawford, Duane). 

Unilateral nystagmus is rare, some fifty-tw T o cases having been 
reported. It is generally vertical, but was horizontal in cases described 
by v. Eeuss, Everbusch, Bouchaud, Nagel, K. Sachs, Norrie (two cases), 
Xeustatter, and Oppenheimer, and in one that I saw in Dr. Arnold 
Knapp's clinic. Unilateral oblique nystagmus has been seen by Xeu- 
statter, and unilateral rotary nystagmus by Weber, Simon (two cases), 
Xeustiitter, and myself. Simon saw one case in which for a time the 
nystagmus was unilateral and mixed (vertical and rotary), but later 
became bilateral and horizontal. 

Sometimes when the nystagmus is apparently confined to one eye, 
it will be found that slight, inconstant oscillations are present in the 
other. These may be discovered only with the ophthalmoscope, which 
reveals a peculiar indistinctness of the fundus due to the tremor. 

In other cases still, as proved by Simon's experience just cited, and 
in one case of mine, a unilateral nystagmus may become completely 
bilateral. 

Finally, just the reverse may occur, — i.e., a nystagmus originally 
bilateral may become confined to one eye. Thus, in spasmus nutans, the 
nystagmus may disappear from one eye before it disappears from both 
(Schapringer) ; and a bilateral nystagmus due to opacities of both 
cornea? may disappear first from the eye which clears the first (Simon). 

Conditions causing Yariatio)is in Nystagmus.- — Xystagmus is usu- 
ally more or less intermittent. And, whether intermittent or not, nys- 
tagmus will in the same individual show considerable variations in 
both the amplitude and the rapidity of the oscillations. These .varia- 
tions are often determined by specific conditions, — e.g., by physical and 
psychical excitation or depression, by sensory stimuli (particularly 



276 THE EYE AND NERVOUS SYSTEM 

changes in illumination), by the use of alcohol, and sometimes even 
by voluntary effort (A. Graefe). Nystagmus is often seen to increase 
if the patient looks aimlessly into vacancy instead of fixing; or it in- 
creases if the eyes are carried in some special direction, ceasing perhaps 
if they are turned in another way. In other cases it ceases when the 
eyes are converged on a near object. Lastly, in some cases a nystagmus 
is regularly developed in one or both eyes as soon as one eye is occluded ; 
in other cases such occlusion puts a stop to a nystagmus that is present 
in binocular vision. 

Nystagmus may also change its direction, — e.g., from vertical to 
oblique or even from vertical to horizontal, when the eyes are turned 
in different ways. 

A careful study of the cases showing these variations will, I believe, 
lead to the conclusion that in a given case of nystagmus the oscillations 
become least in that position of the eyes which for this case involves 
the least strain upon the eye-muscles, or they are least under that con- 
dition of vision (exclusion of one eye, use of both eyes, use of glasses, 
etc.), which implies the least confusion to sight. 

D. Combined Forms of Tremor. — In some cases we find searching 
movements combined with a pseudonystagmus. This would seem espe- 
cially to occur when there is absence of central fixation, and at the 
same time the movements of the eyes are more or less restricted. 

In some cases we may find nystagmus and pseudonystagmus com- 
bined in the same patient. Thus, as in a case of Uhthoff's, there may 
be a continual horizontal nystagmus which becomes coupled with a ver- 
tical jerky pseudonystagmus as soon as the eyes are carried far up or 
down. 

Etiology of Nystagmus and Pseudonystagmus. — Searching Move- 
ments are found in blind eyes and in eyes which from chorioidal 
lesions at the yellow spot or similar causes have lost the power of 
central fixation. 

Pseudonystagmus occurs almost constantly in hereditary ataxia, and 
in this disease it is usually very marked. It also is found very fre- 
quently in multiple sclerosis (in 46 per cent, according to Uhthoff). 
But as it is also present in a great variety of other nervous diseases, 
both functional and organic, and not infrequently, in fact, is found 
in people who are perfectly healthy, it is not of great importance as a 
diagnostic sign. 

Pseudonystagmus in many cases, if not always, signifies some weak- 
ness of the muscles involved in making the special associated movement 
in the course of which it occurs. 



NYSTAGMUS AND PSEUDONYSTAGMUS 277 

Nystagmus is caused by — 

(A) Conditions producing bilateral amblyopia in early infancy 
(optical, infantile, or so-called congenital nystagmus). Such conditions 
are opacities of the cornea, especially from blennorrhoea neonatorum; 
congenital cataract ; hemorrhage, disease or abnormalities of the retina 
andchorioid; total congenital color-blindness, and albinism. Refractive 
errors, even when great, do not usually cause it, although they may 
do so. 

Such nystagmus is probably not often really congenital, but develops 
during the first few months of life. 

It must be remarked that unilateral amblyopia very rarely produces 
nystagmus but tends rather to produce squint. Simon, however, reports 
two cases in which an infantile unilateral nystagmus was caused by 
unilateral opacities of the cornea. It is noteworthy that in one of 
these cases the nystagmus was originally bilateral, and disappeared from 
one eye only when the cornea of that eye cleared up. Later, when the 
cornea of the other eye cleared, the nystagmus disappeared from that 
eye, too. 

The unilateral nystagmus that occurs in an amblyopic eye affected 
with squint (Heimann) is probably not often infantile in origin, but 
is to be regarded as a late acquired optical nystagmus (see under type B, 
infra). 

Sometimes a nystagmus of this form, evidently dating from in- 
fancy, occurs without there being any obvious lesion of the eyes to 
cause it. In such cases, according to A. Graefe, the nystagmus is proba- 
bly attributable to a congenital retinal hemorrhage which subsequently 
cleared up. 

(B) Conditions developing in later life causing constant strain of 
the eyesight from poor illumination or excessive strain of the eye- 
muscles, or usually strain of both kinds combined. 

This variety of late acquired nystagmus is usually due to conditions 
incident to the patient's occupation (hence occupational nystagmus). 
The commonest form of this is miners' nystagmus, occurring in coal- 
miners who work with the eyes directed constantly up and back and 
by the insufficient light of the safety-lamps. The bad air of the mines 
is thought to be an additional factor in causing it. Miners' nys- 
tagmus is characterized by its markedly periodic character and its 
tendency to recur under conditions of reduced illumination, or when 
the eyes are directed up, or as a result of bodily exertion. Another 
feature, distinguishing it particularly from early acquired nystagmus 
(type A) is the fact that the eye movements are very greatly restricted 



278 THE EYE AND NERVOUS SYSTEM 

and the patient finds it hard to keep the eyes in the fixing position 
(A. Graefe). 

Similar to miners' nystagmus is the form occurring in compositors, 
paper-makers, metal-rollers, etc., who work with the eyes in a constrained 
position (Snell, Eversbusch), and in those who work in an insufficient 
light or under other conditions peculiarly trying to the eyes (Erost, 
Clarke). Magnus (cited by Snell) tells of the case of a baby who 
developed nystagmus as the result of constantly looking at a light 
above and behind his crib, and who got well when put in a properly 
lighted room. 

In the same category belong those cases in which optical nystagmus 
develops after the age of infancy as a result of high astigmatism (as 
in a case of mine), or of traumatic cataract (Krauss), or of other con- 
ditions producing marked amblyopia. These cases are rare. They are 
comparatively often unilateral, and then are often, if not always, asso- 
ciated with squint, which may indeed be the cause of the amblyopia and 
the nystagmus (Heimann). 

According to Bar a transient nystagmus may be induced by other 
forms of reflex sensory irritation originating in the eye, — for example, 
by inflammation or foreign bodies of the conjunctiva or cornea. Here 
the impulse for the discharge of the oscillatory movements presumably 
passes through the fifth nerve to the pons, finally reaching the abducens 
and oculo-motor nuclei. It is questionable whether in such cases 
the irritation of the eye is really the cause of the nystagmus, or whether 
it has not simply stirred into action a pre-existing nystagmus tem- 
porarily quiescent. 

(C) Irritation arising from the ear {aural nystagmus). Nystag- 
mus often is produced by some irritation arising from the inner ear. 
Such irritations appear to act by setting in motion the internus of the 
same side as the affected ear and the externus on the opposite side. It 
may be produced by actual disease of the labyrinth, by changes in laby- 
rinthine pressure due to rapid rotation of the body, and by operative 
manipulations carried on in the middle and external ear (probing, 
syringing, etc.) (Eversbusch). Peters thinks that miners' nystagmus 
is of labyrinthine origin. 

According to Schwartze, aural nystagmus is not due primarily to 
heightened labyrinthine pressure, but to irritation of the vestibular 
nerve. 

(D) Nervous Disease. — True nystagmus occurs frequently in mul- 
tiple sclerosis (in 12 per cent, of the cases, according to UhthofT). It is 
also frequent in syringomyelia. 



NYSTAGMUS AND PSEUDONYSTAGMUS 279 

Nystagmus has also been described as occurring often in other ner- 
vous diseases, as meningitis, meningeal hemorrhage, sinus-thrombosis, 
hemorrhage and softening of the brain, tumors of the brain, particularly 
the cerebellum, and pons disease (Gowers). In many of the reported 
cases, however, care has not been taken to distinguish between nystagmus 
and pseudonystagmus. Except in multiple sclerosis and syringomelia, 
and in conditions due to ear trouble, true nystagmus is rare in 
nervous disorders ; occurring in only 3 cases out of 500 examined 
by Uhthoff and in only 1 per cent, of tabics. And even in the cases in 
which it does occur it is often not due to the nervous disease itself, but is 
simply an infantile nystagmus produced by opacities in the media, 
congenital defects, etc. 

We may, therefore, say that a true nystagmus, not due to optical 
defect nor dating from childhood, and not traceable to occupation, as in 
miners' nystagmus, nor to ear disease, is strong presumptive evidence 
either of multiple sclerosis or less probably of syringomyelia. 

A transient nystagmus, often of peculiar form, frequently occurs in 
spasmus nutans (Thompson, Spicer, Schapringer). This may be uni- 
lateral, or be bilateral at one time and unilateral at another. 

Nystagmus may occur in hysteria; and in hysterical subjects has 
been evoked and cured by suggestion (Sabrazes). 

Nystagmus, probably due to central nervous disease, has been occa- 
sioned by traumatism. Such nystagmus is rare. It may be transitory, 
as in two cases described by Norrie. In some instances, • at all events, 
as in those given by Apelt, it seems to be simply the result of a func- 
tional nervous condition (traumatic hysteria). 

Not to be confounded with these cases of traumatic nystagmus are 
those in which an acquired optical nystagmus (see under type B) has 
resulted from the optical defects produced by a traumatism (two cases 
described by Krauss). 

Both nystagmus and pseudonystagmus occurring in nerve disease 
are often associated with paresis or weakness of the muscles. 

(E) Poisons. — Nystagmus is said to occur very often in patients 
that are under the influence of ether. Other agents that very rarely 
produce nystagmus are alcohol, cocaine, sulphonal, arsenic, lead, qui- 
nine, ergot, and sewer gas (Uhthoff). 

(F) Voluntary Nystagmus. — Occasionally nystagmus may be pro- 
duced voluntarily (A. Graefe ; Dodd ; A. E. Davis). Graefe thinks 
that this can occur only in those who were formerly the subjects of an 
involuntary nystagmus, which has disappeared. 

Nystagmus, whether infantile or due to nervous disease, is some- 



280 THE EYE AND NEKVOUS SYSTEM 

times hereditary (Nettleship; McGillivray; Audeoud; see also excel- 
lent summary by Groenouw). 

Site of Lesion in Conditions of Ocular Tremor. — In the infantile 
form of nystagmus, as well as in the form due to occupation (miners' 
nystagmus), and to aural irritation, the tremor is not due to any actual 
lesion of the brain. The contrary has been asserted of miners' nystag- 
mus, but, as A. Graefe says, without sufficient proof. In the nystagmus 
of multiple sclerosis and other organic nervous diseases, lesions have 
been found mainly in the corpora quadrigemina, corpus striatum, resti- 
form bodies, cerebellum, and medulla. It may be noted that experi- 
mental stimulation of the same parts and also of the cerebral cortex 
in animals likewise produces nystagmus. Occasionally the nystagmus 
in nervous disease seems to be produced not by a lesion interfering 
directly with the paths for the associated movements of the eyes, but 
by remote lesions which act by causing variations in the intracranial 
pressure (IThthofT). (See Pages 449 and 472). 

In pseudonystagmus due to organic nerve disease the lesions are 
also in most cases central, although in some instances they may be seated 
in the peripheral nerves supplying the muscles. 

Theory of Nystagmus. — No perfectly satisfactory explanation of 
nystagmus has yet been given. The remarkable regularity and paral- 
lelism of the movements, however, show that in nystagmus we have to 
deal with a perversion of the centres for parallel and parallel-rotary 
movements, and not with peripheral lesions of the muscles themselves 
or their nerves. 

This perverted state of the association centres is evidently produced 
in optical and miners' nystagmus by long-continued abnormal stimuli 
passing up through the visual tract, and in miners' nystagmus also 
through the fifth nerve ; in aural nystagmus by abnormal reflexes pass- 
ing through the auditory nerve to the centres in the pons and perhaps 
also in the corpora quadrigemina; while in nystagmus due to nervous 
disease, the association centres and tracts are probably affected directly. 

Nystagmus in all probability is produced by alternate, just as nor- 
mal fixation is produced by simultaneous, discharges of motor energy 
from the two sides of the brain. That is, when we fix, the eyes are held 
steadily in place, because impulses are sent down at the same time from 
the right angular gyrus (which acting alone would cause both eyes to 
move to the left) and from the left angular gyrus (which acting alone 
would cause both eyes to move to the right). In a person with nystag- 
mus the impulses from the right angular gyrus and from the left 
angular gyrus follow each other in rapid succession. 



NYSTAGMUS AND PSEUDONYSTAGMUS 281 

Nystagmus, then, may be regarded as a kind of imperfect or per- 
verted fixation. Infantile nystagmus, in particular, is an incompletely 
developed fixation — a condition intermediate between the wide, more or 
less irregular, excursions of a congenitally blind eye and the steady gaze 
of an eye which fixes normally. 

This explanation of the nature of nystagmus holds good un- 
doubtedly even for the rare unilateral and dissociated forms. These 
differ from the ordinary bilateral and symmetrical nystagmus only in 
the fact that either the reflex discharges from the association centres, 
which cause the oscillation, are in some way so altered as to affect 
one eye more than the other, or else there is a contributing cause, — 
usually some muscular anomaly, — which being mainly seated in one 
eye makes that eye respond more readily than its fellow to the abnor- 
mal stimuli. 

Symptoms of Nystagmus. — The symptoms of nystagmus are — 

1. Apparent Movement of Objects. — We should expect that the 
rapid oscillation of the eyes in nystagmus would make it seem to the 
patient as if all objects that he looked at were jumping to and fro. And 
in miners' nystagmus and the form due to aural disease this apparent 
movement of objects is a very prominent and distressing symptom. It 
is also often, though not always, present in the nystagmus due to nervous 
disease. Sometimes in these cases the patient not only sees objects 
move but feels that the eye is trembling. 

On the other hand, in optical nystagmus due to infantile amblyopia, 
this apparent movement of objects is very rarely perceived. Sometimes 
in unilateral nystagmus and, under exceptional conditions, in bilateral 
nystagmus it can be evoked (A. Graefe). Even then, however, it seems 
hardly ever to be troublesome. 

2. Reading in Vertical Lines. — That the apparent movement of 
objects, even when it is not perceived by the patient, yet causes indis- 
tinctness of things seen, is shown by the fact that some patients with 
horizontal nystagmus in reading hold the book sideways, so that the 
lines of print run vertically. This, to a certain extent, obviates the 
confusion produced by the oscillations of the letters ( Soelberg- Wells, 
Wecker, Duane). 

3. Vertigo. — This is marked in some of the late acquired forms, 
especially in miners' nystagmus and the form due to aural irritation. 

4. Nodding Movements of Head. — These often occur in optical nys- 
tagmus. They were formerly thought to be compensatory in character, 
acting to prevent the tremor of objects produced by the oscillation of the 
eyes. This, however, is certainly not the case ; indeed, in one instance 



282 THE EYE AND NEKVOUS SYSTEM 

that I saw the nodding of the head considerably increased the confusion 
of sight due to the nystagmus. 

5. Poor Vision. — In most cases of nystagmus of infantile origin, 
the vision is quite poor, and cannot be brought up to normal by glasses. 
This is doubtless in part due to the blurring that the nystagmus pro- 
duces, but much more to the pathological condition causing the nystag- 
mus. In fact, nystagmus in itself does not necessarily cause permanent 
poor sight. I have seen it associated with a vision of nearly f£- 
It is to be inferred that in such cases the application of glasses or the 
mere act of fixing attentively with the moving eye has made the latter 
quiet for the time being, since it is very difficult to suppose that the 
sight could be anything but bad so long as the eyes are still in active 
motion. In fact, we can prove in those cases in which the nystagmus 
increases greatly in one direction of the gaze and diminishes in another, 
that the sight is very much better in that position in which the oscilla- 
tion is slight. In miners' nystagmus the disturbance of sight produced 
by the tremor is considerable. 

G. Photophobia may be present. In unilateral nystagmus it may 
occur only when the oscillating eye is used to see with (Schapringer). 

Course of Nystagmus. — Infantile nystagmus generally lasts through 
life, although it may disappear. Nystagmus due to aural or nervous 
disease naturally persists as long as its cause. Miners' nystagmus dis- 
appears if the patient gives up his work and lives under proper condi- 
tions as regards illumination and eye-strain. Nystagmus due to spas- 
mus nutans and hysteria is probably always transient. A transient 
nystagmus may also result from traumatism (Norrie), tenotomy 
(Wecker), or other causes (Simon, and others). 

Treatment. — The treatment of nystagmus must be directed to the 
cause. Infantile nystagmus is rarely affected by any treatment, 
although isolated cases have been cured by removal of a congenital 
cataract (Crzellitzer, Cuperus), by tenotomy or advancement causing 
relief of a squint (Kugel, Neustatter, Heimann, Cuperus), by exercises 
with a stereoscope and in reading with the aid of a perforated dia- 
phragm (Lavagna), by exercises in fixation in various positions of the 
gaze (Brisseaud; Meige and Feindel), and by exercises with rotating 
prisms (Trombetta). As a temporary help Kugel recommends pro- 
tracted bandaging of the eyes and the use of dark glasses before the 
better eye. Some cases of nystagmus, especially acquired nystagmus, 
are relieved by the correction of astigmatism or other refractive errors 
(Kugel, Duane). In miners' and other forms of occupational nystag- 
mus change of occupation is imperative. 



ANOMALOUS DEVIATIONS 283 

Section VI. 
ANOMALOUS DEVIATIONS OF THE EYES. 
DISJUNCTIVE MOVEMENTS. 

In some cases the eyes no longer follow the law of associated move- 
ments, but each moves irrespective of the other. This occurs, for ex- 
ample, in eyes that have been totally blind from birth, also in patients 
deeply under the influence of chloroform. But it occasionally occurs 
in other cases, too. 

Thus, a deviation of one eye down and out and of the other eye up 
and in is said by Gowers to occur in disease of the middle cerebellar 
peduncle. 

Lawford describes a case in which, when the patient was told to 
look to the left, both eyes moved outwards. (This may have been simply 
an instance of excessive voluntary divergence of the eyes — divergence- 
excess). 

Kunn gives six cases of dissociated paralyses. 

Uhthoff says that occasionally dissociated ataxic movements occur 
in tabes. 

I have seen a case in which the right eye at intervals moved slowly 
up and after awhile moved down again, while the left eye remained 
perfectly still. The movement of the right eye took place whether the 
left eye was covered or open. The condition was probably a sort of 
unilateral searching movement (page 273), and may be regarded as 
an abortive form of unilateral nystagmus. 

A special form of partial dissociation of the ocular movements is 
seen in those cases in which on alternate covering each eye goes up or 
each eye goes down behind the screen. Cases of this sort have been 
described by Schweigger and especially by Stevens. I myself have 
seen three or four. In these cases there may or may not be binocular 
fixation when both eyes are uncovered. If there is binocular fixation, 
the condition is called anophoria or double hyperphoria if both eyes go 
up, and catophoria or double hypophoria if both go down, when 
screened. The corresponding conditions in which one eye squints when 
both are uncovered are called anotropia and catotropia. Such a squint 
may be alternating, and then in anotropia each eye in turn will squint 
up (double hypertropia) and in catotropia each eye in turn will squint 
down (double hypotropia). 

Some of the cases described as illustrating dissociation of the eye 
movements are not really of this character. Thus, Konigshofer's case 
in which, without moving the right eye, the left could be voluntarily 



284 THE EYE AND NERVOUS SYSTEM 

turned in or out or be made to look straight ahead, was evidently one 
of left divergent squint, which was overcome by a strong effort of con- 
vergence, both eyes at the same time being carried to the right by an 
associated parallel movement. Again, the case given by A. Graefe in 
which the left eye went straight up when the attempt was made to move 
both eyes to the right, can readily be explained by supposing that, as 
the left eye could not move inwards because its internus was paralyzed, 
it was forced spasmodically upward by the overaction of its elevators, 
which were brought excessively into play. (Of. remarks, page 249.) 

Weinhold's case, in which a patient with usually normal eye move- 
ments could at will produce a left strabismus sursumvergens, marked 
when he looked to the right but more and more slight when he looked 
to the left, was probably one of moderate insufficiency of the right 
superior rectus. In this case, if the patient fixed with the left eye 
the deviation would be but slight, even when he looked to the right, 
and might indeed be absent altogether if, as could well happen, he had 
developed an excessive ability to diverge the eyes in a vertical direction. 
If, however, by a voluntary transition he fixed with the paretic right 
eye, the left eye would immediately assume a position of marked sec- 
ondary deviation and would shoot up, especially when he looked to the 
right. 

An apparent dissociation of the ocular movements, furthermore, may 
exist in those cases in which several muscles are paralyzed in one eye 
and yet this eye is used for fixation. Here, as A. Graefe points out, the 
secondary deviations of the sound eye, rapidly changing their form and 
varying their amount as the eyes are carried in different directions, 
make it appear as if the eyes were moving quite contrary to the ordinary 
laws of association. 



CHAPTEE VI. 

THE INTRAOCULAR MUSCLES. 

By JOHN E. WEEKS, M.D. 

IRIS. 

The iris is the terminal portion of the vascular tunic. Its free 
border, which describes a circle enclosing the space known as the pupil, 
rests on the anterior surface of the capsule of the lens. As the lens 
surface occupies a plane anterior to the ciliary border of the iris, the iris 
forms a shallow cone, the depth of which depends on the position of the 
crystalline lens. If the lens is absent, the iris hangs in a vertical plane 
and is tremulous. 

The diameter of the iris (in small eyes 9 millimetres, in large eyes 
13 millimetres) is approximately 11 millimetres. The iris is not of 
uniform thickness, but averages 0.04 millimetre when at rest. When 
widely dilated it may double in thickness. 

At the insertion of the iris the posterior two-thirds pass into the 
ciliary body. The anterior third is continuous with the ligamentum 
pectinatum (Compare Page 22). 

Anterior Endothelial Layer. — The iris is limited anteriorly by a layer 
of flat, nucleated endothelial cells, which is continuous over the surface 
of the trabecule of the ligamentum pectinatum and the posterior surface 
of the cornea. 

Vascular Stroma Layer. — The " vascular stroma layer" is composed of 
bundles of connective tissue loosely associated. This layer contains 
numerous blood-vessels, nerve-trunks, and irregular lymph-spaces. 
Round, oval, and branching nucleated cells, the protoplasm of which 
becomes pigmented in colored irides, are found in this layer, as are also 
the muscles of the iris — the sphincter and dilatator pupillse. 

The sphincter pupillae muscle lies very near the pupillary margin. 
It is a flat, circular band, measuring about one millimetre in width, 
varying slightly according to the state of contraction, and is about one- 
tenth as much in thickness. It is composed of bundles of non-striated 
muscle-fibres which interlace. The sphincter pupillse is separated from 
the pupillary margin by the posterior pigment layer of the iris, which 
extends a very little way over the stroma at the pupillary margin and 

is seen as a narrow, black ring at this point. 

285 



286 THE EYE AND KEKVOUS SYSTEM 

The dilatator pupillas consists of two or more layers of long spindle- 
shaped cells possessing elongated nuclei. They are undoubtedly non- 
striated muscle-fibres. These fibres are arranged meridionally and lie 
on the limiting membrane. They apparently form a continuous layer 
extending from the ciliary margin of the iris to the sphincter pupillae, 
with which they blend, entering at the outer border and posterior 
surface. 

Lamina Vitrea, Membrane of Bruch. — This extremely delicate mem- 
brane is homogeneous under low magnifying powers, but presents fine 
striations when examined with high powers of the microscope. It is 
extremely thin (0.002 millimetre) and is closely adherent to the inner 
layer of cells of the pigment layer. 

Pigmented Layer (Uvea of the Iris). — This represents the extreme 
anterior portion of the secondary eye vesicle. The cells of the deeper 
layer are somewhat flattened, are spindle-shaped, and are arranged 
radially to the pupillary margin. They rest upon the lamina vitrea of 
the iris. The cells of the outer layer are short, cylindrical or polygonal 
cells. It is now held by some histologists that the inner layer of cells 
which forms the pars iridis retinae is concerned in the development of 
the sphincter and dilator muscles of the iris. At the ciliary attach- 
ment, the pigment layer of the iris is continuous with the pigmented 
layer of the ciliary body, the pars ciliaris retinae. 

Vessels of the Iris. — The arterial supply to the iris is derived from 
the long posterior ciliary arteries and from the anterior ciliary arteries. 
The capillaries of the iris terminate in the venae radicae which proceed 
radially to the ciliary body ; they empty into the venae vorticosae. The 
walls of the arteries are thick, due to a preponderance of connective 
tissue without elastic fibres. Muscle-fibres are said to be relatively few. 
The arteries and veins possess thick sheaths of connective tissue, between 
which and the vessel wall proper the perivascular lymph-space is found. 

Lymphatics of the Iris. — There are no distinct lymph-vessels in the 
iris. The clefts between the tissue elements of the anterior border 
layer, the irregular spaces between the bundles of fibres of the vascular 
stroma and the perivascular spaces, are the lymph-channels of the iris. 
They communicate with the lymph-spaces in the ciliary body, with the 
lymph-spaces at the filtration angle, and through the spaces of Fontana 
with the anterior chamber. 

Nerves of the Iris. — The bundles of nerve-fibres that enter the iris are 
derived from the ciliary plexus that lies in the ciliary muscle. Three 
kinds of fibres are given off from this plexus: (1) The sensory; (2) 
the motor; (3) the sympathetic. The motor-fibres from the third nerve 



THE INTKA-OCULAK MUSCLES 



28' 



pass to the sphincter pupillse. The sympathetic nerve-fibres pass back- 
ward toward the dilatator and are supposed to supply it (Baker). 

Function of the Iris. — The function of the iris is to regulate the 
quantity of light that enters the eye so that the act of vision will be 
carried on with the greatest possible efficiency. The iris is a diaphragm 
which changes the size of its central opening, the pupil, as the conditions 
demand. The movements of the iris are involuntary and are made 
unconsciously. They are induced by stimuli which act through certain 
" reflex arcs." The stimuli which produce these movements of the iris 
are light, cutaneous sensations, emotions, convergence, accommodation, 



Bulbus sinister 



M. rectus superior 



x Radix brevis seumotoria 



Ganglion Gasseri 




«-. N. infratrochlearis 



N. ethmoidals 



N. ciliares longi 



N. naso-ciliaris 

N". opticus 
Plexus caroticus 



Chiasrna 



Arteria carotis 
Tractus opticus 
N. oculomotorius 



N. trigeminus 

Fig. 1 illustrates the anatomical relation of the short and long ciliary nerves : x, short root of the 
ciliary ganglion ; motor (from the oculomotor nerve) xx, long root of the ciliary ganglion ; sensory 
(from the trigeminus) xxx, sympathetic root of the ciliary ganglion (from the carotid plexus of the 
sympathetic nerve). (Piltz). 

association with certain movements of the eye and eyelids. Stimulation 
of the motor oculi nerves causes contraction of the sphincter iridis with 
consequent narrowing of the pupil. Paralysis of this nerve causes the 
sphincter to relax, dilating the pupil. Stimulation of the ciliospinal 
centre in the spinal cord, or of the superior portion of the cervical sym- 
pathetic, causes contraction of the dilator of the iris, resulting in a 
widening of the pupil to a greater extent than obtains in paralysis of 
the motor oculi. Paralysis of the sympathetic causes a narrowing of 
the pupil (miosis) greater than that which obtains when the third 
nerve is also paralyzed. 



288 THE EYE AND NERVOUS SYSTEM 

Normal Pupil. — The size of the pupil varies greatly in different in- 
dividuals and in different degrees of illumination, ranging from 1.5 
millimetres by bright illumination to 9 millimetres by weak illumina- 
tion. The average diameter in young adult life by moderate illumina- 
tion is 4 millimetres. In infants the pupil is small; in youth, large; 
and in the aged it is again small. Lange * measured 1000 pupils in 
patients of different ages and sex. His conclusions were as follows: 
(1) The size of the pupil differs greatly in different persons. (2) As 
a rule, women have larger pupils than men. (3) The size of the pupil 
decreases with age. (4) The width of the pupil is less in hypermetropic 
eyes than in emmetropes and greater in myopes than in emmetropes. 
(5) After the fortieth year the difference in the width of the pupil in 
different states of refraction is inconsiderable. 

The position of the pupil is, as a rule, a little to the nasal side of 
the centre of the iris. The pupils change in size symmetrically. 
Marked inequality in the size of the pupils is usually a pathological 
phenomenon. Slight differences in the size of the pupils frequently 
exist under normal conditions. Iwanow 2 examined 134 military re- 
cruits, finding an equal width of the pupils in only twelve; the right 
pupil was larger in 49 ; the left in 73. Eelton 3 found an inequality 
of the pupils in 28 of 61 persons examined. 

EXAMINATION OF THE IRIS. 
In the examination of the iris a number of methods are employed. 
1. Simple Inspection. — For this, moderate illumination is necessary. 




Fig. 2.— Priestley-Smith keratometer. 

When the pupils are compared, the illumination of each eye should be 
equal. 

2. Oblique Illumination. — This is of service for the purpose of throw- 

1 Inaug. Dissert., Amsterdam, 1901. 

2 Vratsch, No. 7, 1887. 

3 Inaug. Dissert., Bonn, 1895. 



THE IXTRA-OCULAE MUSCLES 



289 



ing light on various parts of the retina in studying the pupillary light 
reflex. 

3. Lenses. — These aid in determining minute movements of the iris. 
They may be simple or compound. Compound lenses are monocular or 
binocular. A monocular instrument that has given some degree of 
satisfaction is that devised by Howe of Buffalo and manufactured by 




Fig. 3. 



Meyrowitz of Kew York. The monocular instrument is inferior to the 
binocular, since with the binocular compound lenses stereoscopic vision 
is obtained. Jackson's binocular loupe adjusted to a head-band is a 
very good instrument. Another, and probably the best, is the corneal 
microscope of Czapski, made by Zeiss of Jena. 

Methods of Measuring the Iris. — Measurement of the diameter or 
width of the iris may be accomplished by means of a small millimetre 
measure, placing the measure before the eyes as close to the cornea as 




Fig. 4.— Circular pupillometer. 

possible. The width of the iris at its line of insertion at the ciliary 
body is somewhat hidden by the anterior border of the sclera at the 
sclero-corneal margin. This fact must be taken into consideration in 
making the measurements just referred to. The keratometer of Priest- 
ley-Smith (Eigs. 2 and 3) may also be employed for this purpose. 

The size of the pupil may be measured by means of a millimetre 
rule, k but, if this is employed, it must be remembered that the size of 
the pupil is actually greater than it appears to be on the rule and that 
19 



290 



THE EYE AND NERVOUS SYSTEM 



tlie difference in size is in direct proportion to the distance of the rule 
from the pupil, since the observer's line of vision cuts the rule before 
reaching the pupil. A very simple and excellent method is by means 
of the ordinary circular pupillometer (Fig. 4). The little disk is 
held close to the cornea and is rotated until an aperture in the margin of 
the disk corresponds to the size of the pupil. The diameter of this 
opening, which is marked on the disk, is then recorded. 

There are many other pupillometers that may be employed. The 




Fig. 5.— Hirschberg pupillometer. 

pupillometer of Soleberg Wells, of Hirschberg (Fig. 5), of Landolt, 4 of 

Schirmer, 5 etc., but these possess little advantage over the simple one 

first mentioned. 

LIGHT REFLEXES. 

The reaction of the pupil to light is excited (1) by the direct 
entrance of light, known as the direct light reflex; (2) by the entrance 
of light into one eye, the observed eye being shaded — the Indirect or 
consensual light reflex. Both reflexes are due to the integrity of what 
is termed the light reflex arc, which consists of fibres running from the 
eye to the brain, known as centripetal fibres, and fibres running from the 
brain (nucleus of the third nerve) to the iris, centrifugal fibres. 

The centripetal fibres have their origin in the retina (according to 
Coppez 6 in the amacrine cells, but this is not fully proven), being 
apparently most plentiful in the region of the posterior pole, diminish- 
ing in number towards the periphery, apjDarently having the same dis- 
tribution as the fibres concerned in vision, that is, those that decussate 
come from the nasal half of each retina, the non-decussating from the 
temporal half. They pass into the optic nerve and, according to von 
Gudden, von Monakow, Bernheimer, 7 and others, can be differentiated 



4 Landolt, De Wecker et Landolt, Traite complet d'Ophthalmologie, p. 942. 

5 Schirmer, Arehiv f. Ophth., xl. 5. 

6 Coppez, Revue generale, February 28, 1903. 

1 Bernheimer, Arehiv f. Ophth., Bd. xlvii. S. 1. 



PLATE II. 




(Fig. 6. 
Pupillary Light Reflex Arc ; Sympathetic, Cortical, and Sensory Dilator Tracts. 

Red and green.— Centripetal tract of the light reflex arc (crossing at the sphincter centres not 
shown. ) 

Yellow.— Centrifugal tract of the light reflex arc. 

Blue.— Sympathetic tract containing the nerve-fibres which innervate the dilator pupillse. 

Black.— Cortical and sensory tracts. ( Uhthoft*. ) 



THE USTTRA-OCULAE MUSCLES 291 

from the fibres that have to do with vision by their larger size. Passing 
into the chiasm, they there undergo semi-decussation and proceed along 
the optic tracts to the outer geniculate bodies, where, according to 
Bernheimer, they bend inward, pass to the anterior part of the anterior 
corpora quadrigemina, and thence to the sphincter nucleus in the an- 
terior portion of the nucleus of the third. Bernheimer is not positive 
that the centripetal fibres pass into, but thinks that they may end in 
arborations at the sphincter nucleus. Experimental and pathological 
data at hand make it probable that the centripetal fibres of the light 
reflex arc do not pass through the anterior corpora quadrigemina. 
Moeli 8 reports a case of absence of light reflex in which the lesion was 
found to be a tumor involving both sides of the posterior walls of the 
third ventricle, making it probable that the fibres referred to pass in 
these walls. In this case the pupils were 5 millimetres in diameter, 
equal in size; vision, motility of eyes, and convergence (page 301) 
reflex normal. 

The experiments of Ferrier and Turner 9 seem to prove conclusively 
that the fibres do not pass into the corpora quadrigemina. Young 
monkeys were employed. The corpora quadrigemina were exposed by 
opening the skull and removing the occipital lobe of the brain backward 
from the parieto-occipital fissure. The anterior quadrigeminal bodies 
were then destroyed with the cautery. This did not abolish the pupillary 
light reflex. The movements of the iris were affected only when the 
lesion extended into the underlying Sylvian gray matter. Clinicai 
evidence to this effect 10 is also obtainable. 

Bach 1X is of the opinion that the centripetal bundle of fibres divides 
at or near the external geniculate body one part proceeding to a centre 
in the lower part of the medulla, which he terms the inhibitory centre, 
fibres returning from this centre to the sphincter nucleus. This sup- 
position is not proven and in the light of the experiments of Huge, 12 
who divided the medulla some distance above its lower extremity with- 
out abolishing or changing the pupillary light reflex, it is highly prob- 
able that this centre does not exist. 

In regard to the effect of lesions of this tract, the experiments of 
von Bechterew 1 3 on dogs led to the following conclusions : 



8 Moeli, Archiv f. Psychiatrie, Bd. xviii. S. 1. 

8 Ferrier and Turner, Brain, 1901, vol. xxiv. p. 27. 

10 Goldzieher and Bechterew, Archiv f. w. ges. Physiol.. Bd. xxxi.. S. GO. 

11 Bach, Graefe's Archiv, Bd. lvii., H. 2, p. 239. 
u Ruge, Graefe's Archiv, 1902, Bd. liv., H. 3. 

"Von Bechterew, Archiv f. w. £es. Phvsiol., Bd. xxxi.. S. 60. 



292 THE EYE AND NERVOUS SYSTEM 

1. Monolateral lesion of the sphincter nucleus causes loss of the 
light reflex of the eye on the same side and loss of the consensual 
reaction in the opposite eye. 

2. A lesion of one side of the third ventricle induces one-sided loss 
of light reflex with good vision. 

3. A lesion affecting both sides of the wall of the third ventricle 
causes loss of light reflex in both eyes, with normal sight. 

Decussation of the Fibres of the Pupillary Light Reflex Arc. — 
The question of semi-decussation in the chiasm is apparently settled. 
Although there is as yet no positive clinical proof, the experiments of 
Bernheimer are conclusive. Bernheimer divided the tractus of one 
side in monkeys and then found that the pupillary light reflex, both 
direct and consensual, was preserved. Decussation or intercommunica- 
tion at the sphincter nuclei is evident both from clinical and from 
experimental observation. The case of S. Weir Mitchell 14 furnishes 
almost, if not quite, conclusive clinical evidence. The chiasm was cut 
in half sagittally by a tumor which divided all but a very few fibres. 
There was bitemporal hemianopsia with retention of the pupillary light 
reflex. Bernheimer divided the chiasm in monkeys by sagittal incision 
and demonstrated the retention of the pupillary light reflex. Whether 
the pupillary light reflex was preserved in the nasal portions of the 
retina? is not stated. Theoretically, this should not occur. 

Lesions Affecting the Pupillary Light Reflex Arc; Centripetal Fibres. 

1. Lesions affecting the retina or optic nerve on one side producing 
blindness cause loss of direct pupillary light reflex on the affected side 
and consensual light reflex on the opposite side. The consensual reaction 
is preserved on the blind side. With uniform illumination the pupils 
are of the same size, as a rule; in some cases the pupil of the blind 
eye is a trifle larger than its fellow in moderate and bright illumination 
when both eyes are equally exposed. If, by moderate illumination, the 
seeing eye is covered the pupil of the blind eye dilates. If there is 
amblyopia (blindness not complete) the direct pupillary light reflex in 
the affected eye and the consensual pupillary light reflex of the fellow- 
eye are not entirely abolished, but are less active than in the normal 
eye. 

2. Sagittal section of the chiasm will, since the distribution of the 
fibres of the pupillary light reflex in the retina correspond to the distri- 
bution of the fibres concerned in vision, cause loss of direct and consen- 
sual pupillary light reflex in each eye when the nasal half of the retina 

14 Jour, of Nerv. and Ment. Dis., January, 1889. 



THE LNTKA-OCULAK MUSCLES 293 

only is illuminated. 15 Illumination of the temporal halves of the retinas 
elicit both direct and consensual pupillary light reflex. With this lesion 
the pupils are slightly larger than normal. 

3. Sagittal section of the chiasm and lesion of one optic nerve, with 
consequent blindness on one side and temporal hemianopsia on the other 
side, cause complete loss of direct pupillary light reflex in the blind eye 
and of consensual pupillary light reflex in the seeing eye; complete 
loss of direct pupillary light reflex in the seeing eye and loss of consen- 
sual pupillary light reflex in the blind eye when the nasal half of the 
seeing eye is illuminated, and retention of the direct pujDillary light re- 
flex in the hemianopic eye and of consensual pupillary light reflex in the 
blind eye when the temporal half of the retina is illuminated. The 
pupil of the blind eye will be slightly larger than that of the fellow- 
eye by bright and moderate illumination, but of the same size in the 
dark (as may be demonstrated by flash-light photography). 

4. Lesion of the tract us on the right side produces left homonymous 
hemianopsia, with loss of the direct pupillary light reflex in the illu- 
minated eye and loss of the consensual pupillary light reflex in the 
fellow-eye (absence of the Wernicke pupillary light reflex) when the 
right half of the retina of either eye is illuminated. Illumination of 
the left, or seeing half, of either retina elicits direct pupillary light 
reflex of the illuminated eye and consensual pupillary light reflex of 
the fellow-eye. The pupils in this condition are slightly larger than 
normal (other things being equal) by moderate illumination. 

5. Lesion of a tractus and destruction of the same half of the chiasm 
or of the optic nerve on that side produce the same clinical conditions 
as those described in 3. 

6. The lesions heretofore described are accompanied by blindness of 
the corresponding parts of the retina?. If it were possible to destroy by 
lesion the bundle of fibres of the pupillary light reflex arc between an 
optic tractus and the sphincter nucleus, there would be no blindness, but 
there would be loss of pupillary light reflex corresponding to that pro- 
duced by lesion of one tractus if one side only was involved and com- 
plete loss of direct and consensual pupillary light reflex on both sides 
if the bundles on both sides were destroyed. The pupils would remain 
of normal size. Lesions at this point do not abolish the convergence 
light reflex. 

Sphincter Nuclei. — The sphincter nuclei, according to Bernheimer, 



15 In testing the reflex with such a lesion, the difficulty in excluding light | -oc 
page 302. ) from the opposite part of the retina must be borne in mind. 



294 THE EYE AND NERVOUS SYSTEM 

Parlia, Kahler and Pick, and others, lie in the anterior part of the 
nucleus for the third, at the posterior boundary of the third ventricle, 
separated from the nucleus for the ciliary muscle. Bernheimer de- 
scribes a central communication of the two sphincter nuclei, and is of 
the opinion that the communication is by means of processes from the 
neurons of either nucleus. 

Centrifugal Fibres of the Pupillary Light Reflex Arc. — These 
fibres arise from the sphincter nucleus of the side corresponding to the 
iris which they are to innervate, pass into the underlying tissue of the 
pons, where they are joined by fibres from the ciliary nucleus. They 
then pass into the trunk of the third nerve occupying, according to 
Ackerman, the central portion of this nerve-trunk; then by the short 
root to the ciliary ganglion; then by the short ciliary nerves (five to 
seven in number) to the posterior part of the globe, passing through 
the sclera, and, forward between choroid and sclera, into the ciliary 
body, from the anterior portion of which they pass into the iris tissue 
and to the sphincter. 

Lesions of the Centrifugal Fibres. — Since the centrifugal fibres after 
leaving the sphincter nuclei do not decussate, and since they are joined 
by the fibres from the ciliary nucleus soon after their origin and are 
incorporated with the trunk of the third nerve before it leaves the pons, 
it is possible to have an isolated lesion of the pupillary light reflex arc 
only at or very near the sphincter nucleus. Caspar 16 reports a case as 
follows: P., aged 38 years; loss of direct and consensual pupillary re- 
flex with preservation of vision ; pupil responds to convergence. He 
places the lesion between the sphincter nucleus and the point where 
the fibres from the ciliary nucleus join the pupillary light reflex bundle. 
Wilder 17 reports two cases of monocular iridoplegia, in both of which 
there was paresis of accommodation. There was complete preservation 
of convergence pupillary reflex. Such a lesion produces loss of pupillary 
light reflex direct and consensual on the side affected. It produces 
paralysis of the sphincter muscle, but does not affect direct or consensual 
reaction in the fellow-eye. Affections of this bundle in other parts of 
its course are almost invariably associated with other third-nerve paraly- 
ses. If the centrifugal pupillary light reflex bundle on one side only 
is affected, the pupil on the corresponding side is larger than its fellow, 
except in the dark, when (flash-light photography) they are of the same 
size. If the bundle on each side is involved, both pupils are dilated in 



16 Archiv f. Augenheilk., Bd. xxvii. p. 291. 
17 Ann. of Ophthal., April 1897. 



THE INTRAOCULAR MUSCLES 295 

all degrees of illumination. They should be equally so, but Heddaeus 
states that anisocoria sometimes exists. 

Complete section of the third nerve produces total loss of the pupil- 
lary light reflex and consensual light reflex on the affected side. 

Convergence Pupillary Reflex. — When, under normal conditions, 
the eyes change their regard from a distant to a near object, or, in other 
words, when the visual lines are simultaneously deflected from paral- 
lelism to extreme convergence, the pupils contract very markedly. This 
phenomenon is called the convergence pupillary reflex. It is most 
pronounced in eyes whose irides are most active and in those which, 
with normal irides, have large pupils, as in children and young adults. 
In the aged, whose pupils are small, particularly in those whose cervical 
sympathetic nerves are paralyzed, and in tabetic patients, as well as in 
all who possess the Argyll-Robertson pupil, the convergence pupillary 
reflex causes but slight movement of the iris. 

This pupillary reflex is often described as a convergence and accom- 
modation reflex and sometimes as an accommodation reflex. It is very 
easy to demonstrate the fact that the reflex is much more closely related 
to convergence than to accommodation. In many cases it is impossible 
to determine that accommodation influences the pupil at all. The diffi- 
culty in eliminating convergence from accommodation makes it almost 
impossible to determine just what influence accommodation has in the 
production of this reflex, but it is certain that some pupillary reaction 
accompanies accommodation in many individuals. 

The amplitude of the contraction depends on the size of the pupil 
and on the activity of the iris. 

In the emmetropic and hypermetropic eye, the amplitude and 
promptness of the reaction is greater than in myopes. 

In pupils of unequal size, the amplitude of movement is greater in 
the eye with the larger pupil, other conditions being equal. 

If one eye is amaurotic, the fellow-eye being good, the reflex is the 
same. Contraction of the pupil also occurs on convergence if both 
eyes are amaurotic, possibly not to so great a degree as in seeing eyes. 

If there is no reaction of the pupil in convergence, paralysis of the 
sphincter is to be suspected. 

This pupillary reflex disappears, as a rule, with absence of con- 
vergence, whether the absence is voluntary, that is, is not exercised, as 
in cases of loss of one eye, in which case the act of convergence is often 
abandoned after the lapse of some time, or in true absence or paralysis 
of convergence. 

The frequent association of loss of accommodation, paralysis of the 



296 THE EYE AND NEKVOUS SYSTEM 

ciliary muscle and of the sphincter pupillse, with loss of convergence, 
points to the close association of the sphincter and ciliary centres in the 
nucleus of the third with the portion of the Drain controlling con- 
vergence, but no anatomical convergence centre has as yet been demon- 
strated. According to Heddeaus, destruction of the left sphincter 
nucleus with the fibres coming to it and going from it causes paralysis 
of the pupillary light reflex in that eye, but does not cause paralysis of 
the convergence reflex. Division of the third nerve abolishes the con- 
vergence reflex. This makes it probable that the impulses necessary 
for the production of the pupillary light reflex and convergence reflex 
are conducted along separate fasciculi of nerve-fibres coming from 
separate centres. 

Heddaeus holds that this reaction is synergistic with convergence. 
The remarkable experiments of Marian 18 show conclusively that the 
reflex is not necessarily associated with contraction of the interni, but 
do not disprove the necessity for the integrity of the convergence action 
of muscles innervated by the third for its manifestation. Marian, 
operating on the eyes of monkeys, transplanted the tendon of the 
superior oblique to the insertion of the internus and the tendon of the 
internus to the insertion of the superior oblique. Divergence was the 
immediate result, but subsequently the eye could be made to converge 
with its fellow, showing that the convergence does not depend on the 
integrity of the internus. Again, the place of attachment of the in- 
ternus was exchanged with that of the externus of the right eye. On 
movement of both eyes to the right (contraction of both interni) there 
was no contraction of the pupils. The externi were made to take the 
place of the interni and eventually convergence became possible. This 
was accompanied by contraction of the pupil, showing that the centre 
for the interni in the nucleus of the third has no intimate connection 
with the centre for the convergence pupillary reflex. The experiments 
do not disprove the connection of the superior and inferior recti, which 
undoubtedly assist in convergence, with the convergence pupillary re- 
flex. Consequently, Marian's conclusion to that effect, although possibly 
correct, is not warranted. These experiments serve to emphasize the 
fact that little is known of the production of the convergence pupillary 
reflex. Marian thinks it possible that an inhibiting impulse may affect 
the dilator centre and permit of a passive contraction of the sphincter. 
The opinion held by some observers that the convergence pupillary re- 
flex depends on an impulse originating in a cortical centre simul- 

18 Deutsch. Zeitschr. f. Nervenheilk., 1903, Bd. xxiv., !?. 274. 



THE INTRA-OCULAR MUSCLES 297 

taneously with the voluntary impulse for convergence operating through 
the intermediate nucleus of the third nerve may be correct. Loss of 
convergence pupillary reflex in one or both eyes has been observed 
without loss of convergence. 19 This fact implies a centre acting 
either directly to cause contraction of the sphincter pupilhe or indi- 
rectly to inhibit (Marian) the action of the sympathetic on the dilatator 
pupilla?. 

In affections of convergence light reflex, the following condition may 
obtain : 

(a) Paralysis of convergence light reflex on one side without other 
paralyses ; retention of pupillary light reflex direct and consensual, on 
both sides. The pupil on the affected side is usually larger than normal 
by strong illumination. 

(b) Paralysis of convergence light reflex on both sides without other 
paralyses (other reflexes remaining intact). 

Lesions causing paralysis of the third in any part of its course cause 
loss of the convergence pupillary reflex. 

Sympathetic Dilator Tract. — The fibres which innervate the dila- 
tator pupillse have their origin in the ciliospinal centre of the spinal 
cord, situated in the anterior cornu 20 in the lower part of the 
cervical and upper part of the dorsal cord. According to Bach, 
this centre is connected with the sphincter nucleus of the third. A 
centre in the medulla is supposed by Bach to be inhibitory to both of the 
centres with which it is connected, namely, with the ciliospinal centre 
and the sphincter nucleus of the third. Ruge 21 repeated the experi- 
ments of Bach on rabbits and found that after complete division of the 
medulla oblongata perceptible pupillary reaction could be obtained, 
apparently conclusively disproving Bach's idea of an inhibitory pupil- 
lary reflex centre in the medulla. 

Eibres from the dilator centre in the cord pass by the rami coni- 
municantes which pass out of the spinal canal with the seventh and 
eighth cervical and first and second dorsal nerves, and enter the cervical 
sympathetic. The motor fibres reach the superior cervical ganglion, 

19 The writer has recently observed a case in which, with double choked disk 
accompanied by headache, there was preservation of the pupillary light reflex, both 
direct and consensual, no paralysis of accommodation or of the power of convergence, 
but complete loss of convergence pupillary reflex. At the same time there was 
paresis of the external rectus of the right eye. Under mixed treatment the eyes 
resumed the normal condition. This would point to a convergence centre entirely 
separate from the sphincter or accommodation nucleus. 

20 Coppez, Revue gener., February 28, 1903. 

21 Graefe's Archiv, Bd. liv., H. iii., S. 483. 



298 THE EYE AND NERVOUS SYSTEM 

from which similar fibres pass to the internal carotid plexus ; thence to 
the Gasserian ganglion and in the superior division of the fifth into the 
nasal branch and by way of the long ciliary nerves to the globe. These 
nerve filaments pierce the sclera near the optic nerve and pass between 
sclera and chorioid forward into the ciliary body, the outer layers of 
which they pierce, reaching the iris. 

It is probable that an interruption of the pupillo-dilator fibres takes 
place in the superior cervical ganglion, as stimulation by non-electrical 
agents produces dilatation of the pupil, which does not occur on irrita- 
tion of non-sensitive tissues unless the faradic current is employed. 

Anderson, 22 experimenting on cats, after excision of the ciliary 
ganglion, also after section of the short ciliary nerves which he con- 
siders equivalent to section of the third nerve (motor oculi), could not 
obtain a contraction of the pupil by light or by any form of stimulus, but 
obtained marked dilatation of the pupil (1) by stimulating the proximal 
end of the sciatic nerve by means of the faradic current; (2) by pinch- 
ing the skin; (3) by stroking the hair. 

The cord was divided at the level of the first cervical vertebra 
and the medulla and brain destroyed. Faradic stimulation of the 
proximal end of the sciatic nerve produced distinct dilatation after a 
one per cent, solution of eserin had been instilled, demonstrating the 
fact that the dilatation could not be due to inhibition of the pupillo-con- 
strictor tone. Section of the cord below the second dorsal nerves 
abolishes all dilatation reaction to stimuli applied below this point. 
These experiments, which have also been corroborated by others, prove 
that the dilator nerve impulses pass through the centre in the cervico- 
dorsal cord and through the upper cervical ganglion. Parsons 23 has 
shown that stimulation of the cortex of the brain has no effect on the 
pupil after division of the third and sympathetic nerves, but, if the 
sympathetic alone is divided, dilatation may be obtained, which Parsons 
explains as being due to an inhibitory effect on the third nerve. 

The experiments of Budge, 24 Hensen and Volkers (1868), O. Lan- 
gendorf (1864), and others prove that stimulation of the ciliary gang- 
lion and short ciliary nerves causes contraction of the pupil, and that 
stimulation of the long ciliary nerves causes dilatation of the pupil. 
Piltz's experiments demonstrate the fact that the contraction of the 
pupil resulting from stimulation of the short ciliary nerves comes on 
suddenly and subsides suddenly on removal of the stimulus; that on 



22 Journal of Physiol., 1903, 30. 

23 Ibid., 1901, xxvi. p. 366. 

24 Die beweg. d. Iris, Braunschweig, 1855. 



HM^H^^HH 



THE mTRA-OCFLAK MUSCLES 299 

stimulation of the long ciliary nerves the dilatation comes on in about 
two seconds and subsides very slowly on removal of the stimulus. 

The effect on the pupil by stimulation of the sympathetic is to 
produce marked dilatation of the pupil on the side affected, which is 
greater than the dilatation after abolition of the function of the third 
nerve, but is not so great as is obtained by the use of potent mydriatics. 
The dilatation after division of the third on stimulation of the sympa- 
thetic reaches almost, if not quite, maximum dilatation, showing the 
effect of the contraction tone of the sphincter under normal conditions 
of the third. Section of the cervical sympathetic, removal of the 
superior cervical ganglion or section of the long ciliary nerves produces 
contraction of the pupil on the side affected without entirely abolishing 
the pupillary light reflex. The miosis is maximal. 

Braunstein's experiments 25 caused him to conclude as follows : 

1. Stimulation of the outer of the two long ciliary nerves causes a 
•dilatation of the pupil in the outer upper half. 

2. Stimulation of the ocular end of the divided cervical sympathetic 
nerve produces an even dilatation of the pupil, not maximal. 

3. Stimulation of the divided cervical sympathetic after division of 
the outer of the two long ciliary nerves produces dilatation of the inner 
lower half of the pupil (Compare Chapter I, pp. 5, 8). 

Examination of the Light Reflex. 

If the eye, after being covered, is quickly uncovered and exposed to 
moderately bright illumination, the pupil rapidly contracts and reaches 
its minimum size (primary narrowing). There is then a slight dilata- 
tion (secondary dilatation) and perhaps a second slight contraction be- 
fore the size becomes fixed. This phenomenon may be even more plainly 
manifest in the change from parallelism of the visual axes (distance 
vision) to strong convergence (near vision). The oscillation of the 
iris under these normal conditions resembles in small degree the phe- 
nomenon known as " hippus." The primary contraction may occur 
very quickly; indeed, so quickly that it may not be noticed, the second 
•dilatation being the first movement seen. Under these circumstances 
the movement may be readily mistaken for the " paradoxical" pupillary 
reaction. 

Examination for the Direct Light Reflex. — If there is any doubt 
regarding the reaction of the pupil to light, the following method should 
be employed. The patient should be placed in a dark room facing and 
'" fixing" a single source of light. The light should be not more than 

25 Ein BeitrJige z. Kent, d. Innervation d. Irisbeweiningen. Charkow, 1893. 



300 THE EYE AND KEKVOUS SYSTEM 

one metre from the patient. One eye of the patient is excluded from 
the light by covering it with the hand or some opaque object. By means 
of a convex lens (15 to 20 D.) the light is now focussed in the exposed 
eye, the patient not being permitted to look in any other direction than 
in a direct line to the light, The light may be thrown into the eye and 
shut out of the eye at the will of the examiner. The position and 
direction of regard eliminate any influence upon the pupil by accom- 
modation or by convergence. The strong illumination of the eye thus 
obtained makes it possible to determine with certainty the presence or 
absence of the pupillary light reflex. Points to determine are : 

(1) Are the pupils of normal size when the eyes are in shadow and in 
light? 

(See normal pupil.) The pupils may be larger than normal and of 
equal size in shadow if under the influence of a mydriatic. They may 
be smaller than normal and equal in size in light (a) if the irides are 
equally inflamed; (b) if they are atrophic; (c) if there is disease of 
the sympathetic affecting both sides; (d) in senile atrophy; (e) if 
under the influence of a miotic. 

The pupils may be larger than normal when exposed to bright light 

(a) in loss of central acuity of vision and in total blindness due to a 
lesion peripheral to the corpora quadrigemina ; (b) when there is a 
lesion involving the centripetal fibres of the pupillary light reflex arc 
(page 292); (c) in cases of glaucoma with increased tension; (d) 
when belladonna or other mydriatics are being taken internally in 
sufficient quantity or when mydriatics have been instilled into the eyes ; 
(e) when paralysis of the pupillary constrictor fibres exists, or (f) when 
there is a lesion of the pupillary light reflex arc at any point. 

The pupils may be smaller than normal when exposed to bright 
light (a) when miosis has been induced by a miotic remedy, as by 
eserin, pilocarpin, etc. ; (b) when the sympathetic on one or both sides 
is paralyzed; (c) in some cases of inflammation affecting both irides. 

(2) Are the pupils of equal size in dilation and in contraction? 

The pupils will be of unequal size in shadow, the pupil of the affected 
eye being larger (a) when a mydriatic has been used in one eye only; 

(b) when there is paralysis of the centripetal fibres of the pupillary 
light reflex arc (Eig. 6) ; (c) in disease affecting the tissues of 
the irides unequally. In paralysis of the dilator tract (see Fig. 6) 
affecting one side the pupil of the non-affected eye will be the larger. 

The pupils will be of unequal size in contraction by bright light, 
being larger (a) when a mydriatic has been used in one eye; (b) when 
the centripetal light reflex tract on one side has been interrupted ; (c) 






THE IXTRA-OCULAR MUSCLES 301 

in glaucoma with plus tension, affecting one side more than the other; 
(d) temporarily, following a blow on the eve which has produced 
paresis of the sphincter pupillse. 

The pupil will be smaller than normal (a) when a miotic has been 
used in one eye; (b) in paralysis of the centripetal dilator tract affect- 
ing one eye more than the other; (c) in some cases of congestion of the 
iris of one eye. 

Examination for the Indirect or Consensual Light Reflex. — The 
patient may be placed facing a window, one eye being screened in such a 
manner that it will be in shadow, but so that the observer may see the 
iris sufficiently distinctly to note its movements. The exposed eye is then 
alternately covered and uncovered, the movements of the iris of the 
screened eye being observed. On excluding the light from the unscreened 
eye, the pupil of the screened eye should dilate. On re-admitting the 
light to the unscreened eye the pupil of the screened eye should contract. 
The indirect or consensual reaction should equal the direct reaction. 

Instead of using daylight, the patient may be placed in a moderately- 
lighted room with both eyes exposed and the direct reaction be excited 
by reflecting light into one eye by means of a mirror from a source of 
light placed behind the plane of the patient's face. 

The consensual light reflex may be diminished in both eyes (a) when 
the visual acuteness of both eyes is much diminished by disease affecting 
retina, optic nerve, chiasm, or tractus ; (b) when the centrifugal dilator 
tracts are partly, but not completely, jjaralyzed; (c) when there is 
disease of the parenchyma of the irides; (d) when mild mydriatic or 
miotic remedies are instilled into the eye or taken internally in sufficient 
quantity; (e) when the tension of both eyes is slightly elevated. 

The consensual light reflex will be lost in both eyes (a) when there 
is complete blindness due to disease affecting any part of the optic tract 
distal to the point where the centripetal fibres of the light reflex arc leave 
the tract (see Light Reflex Arc) ; 26 (b) when there is paralysis of the 
centrifugal dilator tract on both sides at any point; (c) when the 
sphincter pupillse or dilatator pupillse muscles are paralyzed by the 
use of mydriatics or miotics; (d) when the tension is above normal in 
both eyes. 

The consensual light reflex may be diminished in one eye under 
the same conditions as in diminished light reflex of both eyes when one 
eye or the tracts on one side are affected. 



26 In complete blindness due to double cortical homonymous hemianopsia the 
pupillary light reflex is preserved. 



302 THE EYE AND NERVOUS SYSTEM 

The consensual light reflex will be diminished in the normal eye 
(a) when impairment of vision is due to a lesion of the opposite tract 
distal to the point where the centripetal light reflex fasciculus leaves the 
optic tract. In the affected eye (a) when there is a mild effect from 
the instillation of mydriatics or miotics ; (b) when partial paralysis of 
the dilator tract exists at any part of its course; (c) when paresis of the 
sphincter nucleus or sphincter fasciculus of the third nerve exists; (d) 
when the tension of the eye is greatly increased. 

The consensual light reflex may be lost in one eye (a) when com- 
plete paralysis of the sphincter pnpillse from mydriatics or a blow 
occurs; (b) when the sphincter nucleus or sphincter fasciculus of the 
third nerve is paralyzed and when great increase of tension affects 
one eye. 

In some cases examination by means of photography is desirable, par- 
ticularly, as Schaefer 27 has pointed out, in the cases of anisocoria with- 
out iridoplegia, when almost maximum dilation of both irides simul- 
taneously is recorded. 

The Light Reflex, Direct and Indirect (Consensual), Excited by 
Illuminating the Various Parts of the Retina. — To test the light reflex 
of various parts of the retina, it is necessary to arrange the illumination 
so that with the patient in diffuse light of sufficient brightness to permit 
the observer to discern the movements of the pupils, a pencil of light of 
sufficient intensity may be thrown into the pupil, so directed that only 
the part of the retina to be tested will be illuminated. It is difficult to 
illuminate a particular part of the retina without more or less diffusely 
illuminating other parts, thus making the results of the test unreliable. 
The rays of light should be so directed that they will come to an approxi- 
mate focus on the retina, the refractive error of the patient being taken 
into consideration. The size of the incident cylinder of rays should be 
a little smaller than the pupil. The instrument of von Fragstein and 
Kempner (Fig. 7) may be employed and if properly made is very 
satisfactory. 

Heddaeus advises the use of a very weak source of light, as weak as 
can be employed and enable the observer to see the reaction (a candle- 
or lamp-flame), reflecting the light into the eye by means of the plane 
mirror. In hemianopsia the light should be thrown on the retina as 
peripherally as possible, having the patient rotate the eyes to favor this, 
in order that the sensitive part of the retina may not be illuminated and 
the reflex excited. If the rays of light as they enter the eye are parallel 



27 Dissertation, Giessen, 1899. 



THE INTKA-OOULAR MUSCLES 303 

or nearly so, the part of the retina most intensely illuminated will be 
very small and the best results will be obtained. The Haab reflex 
(page 304) may be employed as a control measure. 

The direct pupillary reflex will fail when light is thrown on that 
part of the retina corresponding to an absolute scotoma in the field of 
vision, occasioned by disease of the retina or disease of the visual tract 
distal to the cerebral end of the pupillary light reflex arc ; that is, in 
cases of disease of the optic nerve, chiasm, or tractus. The pupillary 
light reflex may be diminished in cases in which the blindness is not 
quite complete (the scotoma not being absolute). 

In cases in which the scotoma is absolute, but the lesion in the visual 
tract is situated proximal to the cerebral end of the pupillary light reflex 
arc, other conditions being normal, the illumination of the corresponding 




Fig. 7.— Von Fragstein and Kernpner's apparatus for testing the pupillary reaction. 

blind part of the retina will elicit the direct and consensual pupillary 
light reflex (Wernicke pupillary reaction). 

Method of Testing the Convergence Reaction. — The individual is first 
directed to look at some object at a distance, when the size of the pupil 
is noted. He is then requested to look at a point at a distance of ten or 
twelve inches from the eyes and in the median plane of the head, the 
illumination remaining unchanged. When the gaze is changed from 
the distant to the near-point a contraction of the pupils will occur. If 
now the gaze is again directed to the distant object, a dilatation of the 
pupils will take place. This reaction will take place whatever the degree 
of illumination. If a card of test-type is held before the eyes and 
removed and approached to the eyes, an excellent convergence pupillary 
reflex may be obtained. Individuals who cannot converge and accommo- 
date (small children, the blind, and the insane) do not show the con- 
vergence pupillary reflex. 

Sensory Reflex (Pain Reflex). — Dilatation of the pupil is excited under 
normal conditions by irritating sensory parts in almost any part of the 
body. The reflex path is by way of the sensory nerves to the ciliospinaJ 
centre in the cord and along the dilator tract to the iris. If the skin of 
the neck, particularly the back of the neck, is pinched, pricked, or pulled. 



304 THE EYE AND KEKYOUS SYSTEM 

the palm of the hand or sole of the foot tickled, dilatation of the pupil 
follows. 

The diameter of the pupil changes (dilates) as a result of every emo- 
tion — fright, fear, sudden joy or sorrow, amusement, Bumke 28 attrib- 
utes this to excitation of the cells of the cortex cerebri. 

Irritation of the skin by sudden applications of cold or heat will 
cause the pupil to dilate. 

It has been observed 29 that irritation or stimulation of the pneumo- 
gastric, hypoglossus and laryngeal nerves causes dilatation of the pupil. 

Oculo-pupillary reflex is a term given by von Varady and others to 
the pupillary reflex due to exciting sensory surfaces, particularly of the 
skin and mucous membrane of the lids. Sensory mydriasis is probably 
always followed by comparative miosis. If the excitation is continued, 
mydriasis is again induced, followed by miosis. Yon Yarady found 
that the most practical method was to prick the skin of the eyelid with a 
needle and then observe the pupillary reflex, which is that of dilatation 
followed by miosis. As studied by von Yarady, the reflex differed in 
different individuals. It might be absent in apparently healthy indi- 
viduals, present or absent in functional disturbances or sensibility, 
namely, hysterical anaesthesia or hyperesthesia, but it was never present 
in cases of organsesthesia, affording a diagnostic sign in this condition. 

Haab's Cortical Pupillary Reflex (Attention Reflex). — In 1885 Haab 
( Sitzungbericht d. Ges. d. Aerzte in Zurich, Nov. 21) described a reac- 
tion of the pupil observed under the following conditions : If, when the 
individual is in a dark room, his line of vision being directed forward 
into space or against a dark wall, a lamp or candle is placed at a few 
feet or yards distance (1 metre) at an angle of 45° to the patient's line 
of vision and on the horizontal plane, the reflex will be excited, provided 
the attention (not the line of vision) can be directed to and held upon 
the flame. The reaction is a marked contraction of the pupil which is 
maintained as long as the attention remains fixed on the flame, but is 
relaxed whenever the attention is relaxed. The reflex is marked in 
individuals whose irides are freely responsive to the stimulus of light. 
Some practice is often necessary to elicit the reflex. When the power to 
fix the attention as described is acquired, other luminous sources beside 
the lamp (small window, slit in a blind, etc.) may be substituted if 
desired. Auto-examination may be conducted. 30 The degree of con- 

28 CentralbL f. Nerv. u. Psych., October, 1903, p. 40 

29 Kowalewsky, Investigations on the Innervation in Dilatation of the Pupil, 
Kassan, 1885. 

30 Haab, Archiv f. Augenheilk., Bd. xlvi. 



THE INTKA-OCULAE MUSCLES 305 

traction of the pupil is in direct proportion to the degree of the illumina- 
tion. If a piece of white paper, illuminated by a source of light not 
visible to the patient, be substituted for the lamp, the contraction of the 
pupil is less. If a piece of black paper or other black object be sub- 
stituted, there is no contraction. 

Variation in the position of the source of illumination causes varia- 
tion in the degree of the contraction of the pupil. If the name is carried 
backward beyond 45 p on the horizontal plane, letting the point of fixa- 
tion on the line of vision be represented by zero, the contraction becomes 
less; if in advance of 45°, greater. The reaction also becomes less 
marked if the source of illumination is above or below the horizontal 
plane. 

Both pupils are affected equally with the light falling in one eye. 

The reflex apparently depends on the normal condition of the visual 
fibres throughout corresponding to the side of the retina on which the 
light falls and on the normal condition of the centrifugal fibres from 
the cortex that are concerned in this reflex. According to Haab, the cen- 
tripetal pupillary light reflex fibres, central from the chiasm at least, are 
not necessary (may be destroyed) for the production of this reflex. 

Cortex Reflex. — Stimulation of the cortex has been found to produce 
an influence on the pupil, causing moderate dilatation. Many investi- 
gators have noted the fact that stimulation of the cortex of the brain, 
particularly of the occipital lobe, would cause dilatation of the pupil. 
Stimulation of the anterior four-fifths of the cortex of the cerebellum 
has caused dilatation. 

Parsons, 31 experimenting on cats, dogs, and monkeys, found that 
electrical stimulation of the cortex of the brain " in the neighborhood of 
the crucial sulcus and over a considerable area of the occipital region" 
produced dilatation of the pupil. When the cervical sympathetic was 
intact the pupils became widely dilated, producing " all the usual effects 
of excitation of the nerves themselves." When the sympathetic was 
destroyed the dilatation was not abolished, but was only half or two- 
thirds as great as when the sympathetic was intact. This fact was 
strongly brought out when only one sympathetic was destroyed, the dif- 
ference in the degree of dilatation of both pupils being very pronounced. 
With division of the sympathetic and of the third nerve, stimulation of 
the cortex fails to affect the pupils; they are immobile and remain one- 
half to two-thirds dilated. These experiments lead Parsons to the con- 
clusion that cortical stimulation produces dilatation of the pupil both by 



Royal London Hospital Reports. June, 1904, p. 20. 
20 



306 THE EYE AND NERVOUS SYSTEM 

stimulation of the sympathetic and by inhibition of the sphincter centre 
when the cervical sympathetic is intact, and by inhibition of the sphinc- 
ter centre when the cervical sympathetic is destroyed. 

Braunstein 32 obtained similar results, but attributed them wholly to 
inhibition of the third nerve. 

Evidence of obtaining contraction of the pupil by stimulating cer- 
tain parts of the cortex is not wanting. Schaefer induced strong con- 
traction of the pupil by stimulating the quadrate lobule in monkeys, and 
Terrier by stimulating the middle of the convexity of the hemispheres 
of the brains of pigeons. 

Changes in the size of the pupil by the will is claimed by some to be 
possible. I have myself observed a patient who, while standing before 
a window, looking forward into space, caused his pupils to dilate and 
contract. However, it is so difficult to dissociate accommodative effort 
in these cases that it is not possible to state definitely that these move- 
ments of the pupil are purely voluntary. 

Orbicularis or Lid Reaction (Gifford-Galassi ; Westphal-Piltz). — This 
was first described by Von Graefe 33 as an accompaniment of a general 
contraction of ocular muscles excited by contraction of the orbicularis. 
The reaction has since been described by many writers. By forcibly 
closing the eye it often occurs that the pupil contracts. If the lids are 
held open by a speculum or by other means and an attempt is made to 
close the lids, the pupil in many individuals will contract. 

Harold Giff ord, in writing of this reflex, expresses the opinion that 
it is the result of an overflow stimulus passing from the nucleus of the 
facial to the nucleus of the third nerve, along the longitudinal fasciculus. 
This view is also held by other writers. The reflex may be obtained in 
normal individuals, but often fails. As a rule, in cases of tabes with 
failure of reaction to light but with retention of convergence and ac- 
commodation reflex, the orbicularis reflex is present and is often more 
energetic than the convergence reflex (Westphal and others). UhthofT 
has observed cases in which, with preserved convergence reflex, the 
orbicularis reflex has failed, and, conversely, cases with loss of con- 
vergence reflex in which the orbicularis reflex was preserved. Piltz 
found the orbicularis reflex preserved in 41 to 43 per cent, of the cases 
of tabes. Similar observations have been made in cases of progressive 
paralysis. 

Gilford 34 writes that the reflex is most marked in individuals whose 

32 Zurm Lehre v. d. Inner, d. Pupillenbewegung, Braunschweig, 1894. 
^Graefe's Archiv, Bd. i., H. 1, p. 318. 
54 Archives of Ophthal., vol. xxiv. p. 402. 



THE INTKA-OCULAK MUSCLES 307 

vision is poor and in eyes that are almost blind. While he does not 
ascribe much of diagnostic value to the reflex, he suggests that it may be 
of use to determine whether the sphincter is paralyzed as in cases of 
disease of the nucleus of the third nerve or in brain tumor ; also possibly 
to determine whether a dilatation of the pupil is due to a mydriatic in 
doubtful cases. GifTord is of the opinion that the reaction depends 
chiefly on the palpebral portion of the orbicularis palpebrarum muscle 
and that if, as Mendel believes, the nerve supply to this part of the 
orbicularis comes from the posterior part of the third nucleus, the 
influence on the sphincter pupilla? is easily explained. 

Pupillary Reflex on Movement of the Eyes. — The size of the pupil is 
reduced in many cases by forcible abduction. Ilhthoff 35 has observed 
this reaction in some cases of Argyll-Robertson pupil when the patient 
rotated the eye outward forcibly. Contraction of the pupil by forcible 
rotation outward has been observed by other writers, ~No pathological 
or diagnostic importance has been attributed to it. 

PATHOLOGICAL VARIATIONS IN THE PUPIL. 

The possible pathological variations in the pupil may be stated as 
follows (UhthofT) : 

1. Loss of the pupillary light reflex with retention of the con- 
vergence and accommodation reflex. (Argyll-Robertson pupil.) 

2. Loss of convergence and accommodation reflex and retention of 
light reflex. 

3. Loss of the pupillary reflex for light, convergence, and accommo- 
dation. 

4. Ophthalmoplegia interna, loss of all reflex movements of the 
pupil. 

5. Loss of direct light reflex in an amaurotic eye and consensual 
light reflex in the fellow-eye, due to loss of vision from a lesion periph- 
eral to the chiasm (" reflextaub," Heddaeus). 

6. Loss of sensory or psychical reflex action. 

7. Abnormal miosis with retention of light and convergence reflexes, 
either from abnormal stimulation of the sphincter pupilla?, which may 
include the so-called myotonic pupillary reaction, or from paralysis of 
the dilatator pupilla?. 

8. Abnormal dilatation of the pupil with retention of the light 
and convergence reflex, stimulation of the dilatator pupilla? ( spastic 
mydriasis). 



^Graefe Saemisch, 2 Aufl., Bd. xi., H. ii.. p. 242. 



308 THE EYE AND NERVOUS SYSTEM 

9. Anisocoria (difference in size of pupils). 

10. Changing anisocoria. 

11. Irregular form of the pupils. 

12. Hippus. 

13. Paradoxical pupillary reflex. 

14. Hemianopic pupillary reaction. 

Argyll-Robertson Pupil. — Loss of the pupillary light reflex, either with 
miosis (spinal miosis, paralysis of the sympathetic, absence of dilator 
nerve impulse) or without miosis (sympathetic not paralyzed, skin re- 
flex retained), constitutes Argyll-Robertson pupil. Both irides are 
similarly affected in the greater number of cases, but in some cases (13 
per cent., Rochon-Divignand and J. TIettz) it is unilateral for a long 
time. In quite a large percentage of cases the pupillary light reflex is 
lost in one eye and only partly lost in the other. 

Miosis is present in almost all tabetic cases. 

Paralysis of accommodation of one or both eyes may accompany the 
Argyll-Robertson pupil. 

The convergence pupillary reflex is absent in a small percentage of 
the cases of loss of pupillary light reflex. Moeli puts it at 1 per cent. ; 
Uhthoff states that the proportion between the cases with loss of pupillary 
light reflex only and those with loss of convergence light reflex also, is 
3.5 to 1. Argyll-Robertson pupil has been observed most frequently in 
tabes, where, according to Gowers, it is present in 80 per cent. ; Stern, 
90 per cent. ; Uhthoff, 67 per cent, of the cases. The pupils are of the 
same size in both eyes as a rule. Of the cases observed by Uhthoff the 
pupils were unlike in size in 25 per cent. 

The Argyll-Robertson pupil never occurs in healthy individuals. In 
166 cases of loss of pupillary light reflex, Uhthoff 36 found 67.6 per 
cent, associated with tabes, 8.8 per cent, with dementia paralytica, 8.17 
per cent, with syphilis without tabes, 5.87 per cent, with other cerebral 
diseases, 1.4 per cent, with multiple sclerosis, 2.0 per cent, with railway 
spine and injury of the head, 0.7 per cent, with congenital feebleminded- 
ness, 0.7 per cent, with hystero-epilepsy, 0.7 per cent, with abuse of 
tobacco, 0.7 per cent, with hemianaBsthesia dextram, 3.6 per cent, with 
no assignable cause. It has been observed in hypertrophic peripheral 
neuritis (Dejerine), in poly encephalitis hemorrhagica, and in poly- 
neuritis alcoholica. 

Uhthoff 37 has collected the statistics of Moeli, Siemerling, and 

38 Berlin, klin. Woch., 1886, No. 3. 

37 Graefe Saemisch, Handbuch, 2d ed., chap. xxii. p. 223. 



THE INTRA-OCULAE MUSCLES 309 

Thomsen, embracing 4000 cases of diseases of the nervous system (in- 
mates of asylums). Absence of pupillary light reflex was found in 492, 
distributed as follows : Progressive paralysis, 85.5 per cent. ; tabes, 4.25 
per cent. ; syphilis, 1.83 per cent. ; multiple sclerosis, 1.2 per cent. ; 
injury to head, 0.6 per cent. ; paranoia and epilepsy, 2 per cent. 

Loss of pupillary light reflex is, as a rule, permanent, since it occurs 
the greater number of times as an accompaniment of diseases of the 
nervous system that are progressive. It is sometimes present in cases 
of disease of the nervous system which tend to recover; under these 
circumstances the pupillary light reflex may be re-established. Sulzer 38 
observed loss of pupillary light reflex in the early stage of syphilis in 
11 of 53 cases examined. He followed the pupillary anomaly in some 
of the cases, ascertaining that the condition may develop within three 
months after the primary sore. He states that in general the loss of 
pupillary light reflex in such cases is transitory. 

It is held by some writers that the Argyll-Robertson pupil is inter- 
mittent in some cases. Tanzi 39 reports such a condition in a case of 
progressive paralysis. LThthoff is of the opinion that it does not occur 
in tabes, but thinks that it may occur in less confirmed pathological 
conditions of the nervous system. 

Loss of pupillary light reflex is recognized as often being a very 
early symptom of tabes. 

Dejerine, 40 Dufour, 41 and Uhthoff are of the opinion that all cases 
of Argyll-Robertson are due to syphilis. This view is combated by 
others. Jaffrav has observed it in aortic disease. P. Maria regards this 
form of pupil as an evidence of disease of the posterior columns of the 
cord which may or may not be syphilitic. 

Seat of the Lesion. — It has been stated that one-sided loss of pupil- 
lary light reflex occurs and that in certain cases loss of convergence reflex 
and loss of the power of accommodation occur ; also that miosis is most 
frequently present in loss of pupillary light reflex. Heddaeus is of the 
opinion that the lesion in cases of one-sided loss of pupillary light reflex 
affects the centrifugal fasciculus of nerve-fibres which proceed from the 
sphincter nucleus, the fasciculus for the accommodation reflex remaining 
uninjured. If the fasciculus from the accommodation nucleus is in- 
volved, there is total one-sided iridoplegia. The consensual reaction of 
the fellow-eye is not affected. Others regard the one-sided loss of pupil- 

38 Ann. de Dermatol, et de Syph., March. 1901. 

39 Riv. di patol. nerv. e. ment.. iv. S. 385. 

40 Soc. de Neurologie de Paris, June 15, 1902. 

41 Bull, de la Soc. med. des Hopitaux de Paris, January 13. 1902. 



310 THE EYE AND NERVOUS SYSTEM 

lary light reflex as a nuclear affection. Uhthoff is of the opinion that 
lesion of the portion of the centripetal pupillary light reflex fibres lying 
between the tractus or external geniculate body and the sphincter nucleus 
has more in its favor. If there is a lesion of this bundle on both sides 
at this point, the light reflex will be abolished. Miosis might be pro- 
duced by irritation of the sphincter nucleus, an extension of the process 
to this centre, but Uhthoff believes that the miosis is due to a lesion of 
the cervical sympathetic. 

Pathological research in cases of loss of pupillary reaction has 
afforded but little light on the subject. Pineles, Zeri, and others, in 
examining microscopically the brains of tabetics, found pathological 
changes in the floor of the aqueduct of Sylvius. Round-cell infiltration 
was found in this locality by Kostenitsch. In the examination of 20 
cases of progressive paralysis with Argyll-Robertson pupil, Schutz 42 
found pathological changes in the nerve-fibres in the gray substance in 
the floor of the aqueduct (sclerosis) to which he attributed the loss of 
the pupillary light reflex ; however, he was not able to demonstrate any 
particular bundle of nerve-fibres as being concerned in this degeneration. 
Siemerling and Boedeker have met with similar changes, also with 
minute hemorrhages in this tissue. The cases of Moeli (page 291) 
and Monakow, who found in a case of one-sided loss of pupillary light 
reflex a small sclerosed focus in the tissue between the sphincter nucleus 
and the external geniculate body, apparently involving the centripetal 
pupillary light reflex bundle as it passes from the tractus to the sphincter 
nucleus, support Uhthoff's position. 

A number of investigators refer the lesion to the cervical portion of 
the spinal cord. Wolf 43 found that in cases of tabes and also general 
paralysis in which the light reflex was lost and the knee-jerk intact, the 
lesion affected the posterior columns of the cervical cord. In cases in 
which the pupillary light reflex was retained and the knee-jerk was 
lost, the cord was affected below the third or fourth dorsal vertebra. In 
cases in which both pupillary light reflex and knee-jerk were lost, the 
entire cord was affected. Others have made similar observations. These 
investigators have failed to examine the brain tissue in the floor of the 
third ventricle to determine whether it was or was not involved. Cases 
of loss of pupillary light reflex with intact cord have been reported 
(Dejerine) and cases of cervical tabes with retained pupillary light 
reflex have also been reported (Buck). 



42 Archiv f. Psych., Bd. xxii., H. 3, S. 527. 

43 Ibid., xxxii., H. i., S. 57. 



THE LNTKA-OCtTLAE MUSCLES 311 

Recently Marina 44 has examined the ciliary ganglion and ciliary 
nerves in cases of loss of pupillary light reflex and claims to have found 
pathological changes in all of the cases. He is of the opinion that the 
seat of the lesion is in these structures. 

Ruge advances the hypothesis that disease of the ciliospinal centre 
is followed by atrophy of sympathetic nervous elements in the ciliary 
ganglion, causing an interruption in the reflex arc. The reflex irido- 
plegia of tabes is brought about in this way, making a double lesion 
unnecessary to explain the phenomenon. 

Clinical and pathological evidence regarding the site of the lesion 
in loss of pupillary light reflex to the present time fails of positive 
localization. The weight of evidence indicates a lesion in the vicinity 
of the nucleus of the third and the occasional accompaniment of paralysis 
of extrinsic eye-muscles as well as the ciliary muscles makes the supposi- 
tion plausible. 

Loss of Convergence Accommodation Reflex and Retention of Light Reflex. 
— This condition is rarely met with. It occurs in most cases of con- 
genital absence of convergence, in some cases in which the power of 
convergence is lost; also in some cases in which the convergence is 
abandoned, as in loss of the vision of one eye or in conditions in which 
one eye is not used. It has been observed in tabes, but must be ex- 
tremely rare, as Uhthoff writes that he has never seen it. The author 
has observed it in syphilitic basal meningitis, and it has been seen in 
progressive paralysis, myelitis, and tumor of the corpora quadrigemina. 

The site of the lesion is supposed to be in the brain tissue imme- 
diately below the sphincter and accommodation nucleus, so placed as to 
involve the fasciculus of fibres which, according to Heddaeus, joins the 
convergence centre with the oculomotor nerve. 

Loss of convergence pupillary reaction may affect one pupil alone or 
both pupils. The latter is by far the more common. The convergence 
reaction is seldom absent in tabes, but it occurs in some cases. Sulzer 45 
reports two cases of absence of convergence and light reflex in 53 cases 
of early syphilis. Moeli 46 reports it in one per cent, of 500 cases ex- 
amined. Uhthoff 47 found absence of the convergence pupillary reflex 
in syphilis, 8 cases; tabes, 3 cases; progressive paralysis, 2 cases; 
injury, 2 cases ; " cold," 1 case ; tuberculosis of the brain, 1 case ; 
tumor of the brain, 1 case ; no assignable cause, 12 cases. Convergence 



44 Ann. d. Neurologie, vol. xix. p. 209. 

45 Ann. de Dermatol, et de Syph., March, 1901. 
"Centralbl. f. prakt. Augenheilk., 1885, p. 272. 
47 Berlin, klin. Woch., 1886, No. 3. 



312 THE EYE AND NEKVOUS SYSTEM 

pupillary paralysis has been observed also after diphtheria, alcohol 
intoxication, tuberculosis, Basedow's disease. 

In some of the cases in which convergence and accommodation pupil- 
lary reaction is lost, mydriasis is present, even when the power of 
accommodation is preserved. 

Ophthalmoplegia interna is a term given to the condition of isolated 
paralysis of the sphincter pupillse and the ciliary muscles without in- 
volvement of- the extrinsic eye-muscles. The lesion in these cases is 
without doubt nuclear. It is most frequently unilateral, but may be 
bilateral. Not infrequently the sphincter will be involved first, the 
ciliary muscle later ; the reverse may occur. In rare cases there may be 
isolated paralysis of one or the other muscle. 

In regard to the cause, UhthofT has collected 30 cases. Syphilis was 
the cause in 23.3 per cent. ; tabes, 10 per cent. ; progressive paralysis, 
6.6 per cent; traumatism, 6.6 per cent; cold, 3.3 per cent; intra- 
cranial disease, 6.6 per cent. ; cause unknown, 40 per cent. The pro- 
portion of unilateral to bilateral was approximately 2 to 1. Syphilis is 
by far the most frequent cause. In six cases observed by the writer a 
history of syphilis was obtained in all, and in all the primary syphilitic 
lesion had occurred many years before. Post-diphtheritic ophthalmo- 
plegia interna has been observed. 

In quite a high percentage of cases other portions of the nucleus of 
the third nerve and the nucleus of the fourth and sixth nerves become 
involved. The condition of partial or complete ophthalmoplegia externa 
develops. At times paralysis of the intrinsic muscles follows paralysis 
of the extrinsic muscles. 

Prognosis. — Unilateral ophthalmoplegia interna may remain for 
many years unchanged, but the greater number of cases recover partly 
or wholly or are complicated by involvement of other parts of the central 
nervous system. Double ophthalmoplegia interna is not as a rule so apt 
to remain unchanged. 

Loss of sensory or psychical reflex action will occur in any case in 
which there is a lesion affecting the dilator pupillary tract Destruction 
of the ciliospinal centre, with consequent miosis ; of the superior gang- 
lion of the cervical sympathetic, which produces the symptom complex 
of ptosis, miosis, enophthalmus, and reduction in the tension of the 
globe; of the centrifugal dilator tract distal to the ganglion with the 
consequent miosis, all produce loss of sensory and psychical reflex, with- 
out abolishing the light reflexes. 

Loss of the sensory reflex occurs in all cases of tabes with miosis and 
Argyll-Eobertson pupil. It is in fact absent in all individuals who from 



THE ISTTKA-OCULAR MUSCLES 313 

any cause present miosis with loss of pupillary light reflex. It may also 
be absent in case's of miosis with retention of the light reflex. When this 
occurs, lesion of the dilator tract in some part is the cause. Aside from 
tabes, this condition occurs in progressive paralysis, some cases of 
syphilis, multiple sclerosis, and traumatism. 

This reflex is retained in all normal individuals and in those with- 
out miosis in whom the pupillary light reflex is retained. 

In cases of miosis with loss of sensory reflex, mydriatics effect a 
partial dilatation of the pupil, probably by direct effect on the dilator 
muscle-fibres. 

Abnormal miosis with retention of light and convergence reflex may 
occur from abnormal stimulation of the sphincter pupillse or from 
paralysis of the dilatator pupillse (paralysis of the sympathetic). 

Stimulation miosis (spastic miosis) is frequently accompanied by 
spasm of accommodation. This may be brought about by hemorrhagic 
encephalitis, brain tumor, or abscess which does not involve the sphincter 
nucleus, but simply stimulates or irritates the neurons of this group of 
nerve cells. If the brain disease advances and destroys the sphincter 
nucleus, dilatation supersedes miosis. Spastic miosis may be due to the 
influence of light in individuals with hypersensitive retina?, as in neu- 
rasthenia and in hyperemia of the retina and optic nerve, and, accord- 
ing to Heddaeus, in the first stages of alcoholic amblyopia, abnormal use 
of the eyes and as a result of excessive sexual indulgence. Excessive 
synergistic contraction of the pupils, occurring in hypermetropes who 
are obliged to converge excessively in order to accommodate sufficiently, 
especially in the early stage of presbyopia, may produce spastic miosis. 
This may also occur in emmetropic eyes in cases of weakness of the 
interni. 

Except that produced by miotics, spastic miosis results in narrowing 
the pupil to a greater degree than anything else. The pupil contracts 
very slightly to light and accommodation and is influenced to some de- 
gree by miotics. It differs from paralytic miosis in that mydriatics 
cause dilatation ad maximum. 

Cases of alternating spastic miosis and mydriasis have been reported 
by Bielschowsky, 48 in one of which alternating miosis and mydriasis 
occurred in the right eye at intervals of twenty to thirty seconds. There 
was right partial oculomotor paralysis. When mydriasis was present, 
there was slight divergence. When miosis came on, the eye turned 
inward and downward. 

48 Klin. Monatsblatt f. Augenheilk., Bd. xvi. p. 308. 



314 THE EYE AND NERVOUS SYSTEM 

The myotonic pupillary reaction of Saenger 49 is closely allied to spas- 
tic miosis. This reaction is in the nature of a tonic spasm and may be 
excited by light, convergence and accommodation, and by the orbicu- 
laris reflexes. It is characterized by a very slow recovery from the mio- 
sis occasioned by the stimuli mentioned. When the stimulus is removed, 
the dilatation of the pupil, which normally follows the reflex miosis very 
rapidly, takes place slowly ; that is, in from one-half to ten minutes. 

The phenomenon has been observed only in patients with disease of 
the nervous system. Saenger observed it in a case of suspected tabes. 
The pupillary light reflex was lost. The patient was conscious of the 
excessive contraction of the pupil, he experienced a strained or cramped 
feeling in the temple and forehead. Distance objects were blurred at 
this time, indicating spasm of accommodation. Piltz has observed the 
myotonic pupillary reaction in general paresis with loss of pupillary 
light reflex. 

Saenger is of the opinion that the site of the lesion is in the iris and 
that the condition is analogous to Thomsen's disease. 

Abnormal dilatation of the pupil with retention of the light and conver- 
gence reflexes (spastic mydriasis) is met with in cases of stimulation of 
the cervical sympathetic, whether due to inflammatory processes affect- 
ing the ciliospinal centre or superior cervical ganglion or by sensory or 
psychical influences. It may accompany intracranial effusions and 
intracranial tumor. 50 Abnormal dilatation has been observed after epi- 
leptic seizures, premonitory to locomotor ataxia, in acute mania, and in 
melancholia. Bumke 51 observed excessive dilatation of the pupil in 
fifteen cases of catalepsy, with retention of light and convergence re- 
flexes, in marked anaemia, and sometimes in imbeciles. It is often 
present in cases of intestinal irritation, as from helminths. The pupils 
are dilated and fixed in amaurosis, in complete paralysis of both third 
nerves, and when mydriatics have been used. 

Anisocoria. — Difference in the size of the pupils is frequently ob- 
served. Slight differences may exist under perfectly normal conditions 
(page 288). Pronounced differences in the size of the pupils is 
pathological almost without exception. Difference in the size of the 
pupils, the abnormal pupil being smaller than its fellow, will occur in 

49 Neurolog. Centralbl., vol. xxi. p. 837. 

50 When in the course of intracranial disease of any kind, miosis gives way to 
mydriasis, the symptom is one of grave import, as it signifies partial or complete 
paralysis of the sphincter nucleus either by direct involvement or by excessive 
pressure; paralyses of other branches of the third nerve often accompany or follow 
this condition. 

51 Centralbl. f. Neur. u. Psych., October, 1903, p. 41. 



THE INTRAOCULAR MUSCLES 315 

paralysis of the sympathetic affecting one side, when a miotic has been 
used, when there is congestion of the iris. 

The abnormal pupil will be larger than its fellow when there is 
stimulation of the sympathetic on one side, when a mydriatic has been 
used in one eye only, when there is paralysis of the centripetal fibres of 
the pupillary light reflex arc, after a blow that has produced paresis of 
the sphincter pupilla?, in increase of intra-ocular tension affecting one 
eye more than the other, in paralysis of the motor oculi, in disease of 
the nucleus of the third nerve affecting the sphincter portion. 

Inequality of the pupils " may lead one to suspect locomotor ataxia, 
general paralysis of the insane, unilateral lesion of the third or of the 
sympathetic nerve, pain in a branch of the fifth nerve, carotid aneurism 
or tumor of the neck, unilateral cranial lesion, unilateral acute glau- 
coma." 

A difference in the size of the pupils is common in tabes. Of 166 
cases examined by Uhthoff, 25 per cent, presented a difference in the 
size of the pupils. In 25 per cent, of the cases of syringomyelia the 
pupils are uneven, due to involvement of the ciliospinal centre. Schau- 
man 52 has noted a difference in the pupils of 723 subjects. The num- 
ber includes 401 subjects, or 55.5 per cent, with no organic lesions 
but some neurosis, including 11 with exophthalmic goitre, 4 epileptics, 
and 3 with chorea. In the remaining 322, or 44.5 per cent., there 
were organic lesions, including tuberculosis in 6.8 per cent, and some 
vascular affection in 5.5 per cent. When anisocoria is not due to an 
organic lesion, it is the expression of an asthenic diathesis or a sign 
of hereditary degeneracy. 

Irregularity of the Pupils. — Piltz 53 observed in a new-born child that 
the pupil was not circular, but that it soon became so. He concludes 
that synergetic action of the various parts of the iris does not obtain 
at birth, but soon asserts itself. 

When irregularity of the pupil is observed, it is advisable to use a 
mydriatic for the purpose of excluding synechia?. Irregularity of the 
pupil consists in irregularities in the margin of the pupil due to de- 
fective innervation of the various parts of the muscles of the iris. 
Thus lesion of the outer branch of the long ciliary nerves causes con- 
traction of the upper outer half or quadrant of the iris and a flattening 
of the pupil in that part. The inner branch of these nerves supplies 
the lower inner half of the iris. The various nerve-fibres from the 
sphincter nucleus are distributed to the various parts of the sphincter 

"Abstract, Journal Am. Med. Assc, July 11, 1903, p. 143. 
53 Neurol. Centralbl., 1903, vol. xxii. p. 662. 



316 THE EYE AND NERVOUS SYSTEM 

pupillse and lesion of a part of these fibres would cause paralysis of 
portions of the sphincter pupillse only. 

Piltz divides irregularities of the pupil not due to synechias into 
two classes — (1) changing; (2) constant. 

Changing irregularities present the following conditions. When 
the pupillary light reflex is tested, some parts of the iris contract while 
other parts do not. In a few days the part that was stationary is 
active, the other parts becoming stationary. Piltz has observed this 
form in progressive paralysis and in katatonia. He observed a case 
in which on the 21st of January, 1899, the right pupil was eccentrically 
displaced downward and outward; on the 24th of January the same 
pupil was displaced upward and outward. The pupillary light reflex 
was lost in both eyes and convergence reflex reduced. The orbicularis 
reflex was preserved to some degree. Piltz is of the opinion that 
changing paralysis is due to a shifting of the lesion in the nucleus. 

Fixed irregularities are due to lesions in the nerves that innervate 
the iris that are more or less constant. Piltz observed seven cases of 
progressive paralysis, with irregular pupils in six. The inmates of a 
Swiss asylum for the insane showed irregular pupils in 42 per cent. 
Of 62 cases of tabes, 29 per cent, presented irregular pupils. Intra- 
ocular conditions not infrequently cause irregularity of the pupil, and 
intra-orbital conditions may cause injury of a long or short ciliary 
nerve or destruction of the ciliary ganglion, but these cases are very 
rare. JofTroy 54 states that within the last ten months preceding the 
writing of his article he had not seen a case of Argyll-Robertson pupil 
that did not show some irregularity of the margin of the pupil. He 
regards the condition as often the first sign of advancing disease of the 
innervation of the iris which eventually goes on to complete loss of the 
pupillary light reflex (Argyll-Robertson pupil) and in some cases to 
ophthalmoplegia interna. Piltz has observed that irregularities in the 
pupillary margin are of frequent occurrence in progressive paralysis; 
that in incipient progressive paralysis it is at times the first and only 
symptom ; and it is sometimes the very first sign of the development of 
Argyll-Robertson pupil. The changes observed indicate that they are 
due to disease of the nerves supplying the iris or to their nuclei. 

Hippus. — Under certain conditions, especially when both eyes are 
shaded from the light and then quickly uncovered, the patient being 
placed before a window or other source of light, the pupils will alter- 
nately dilate and contract. This change in size of the pupil under 

54 Rev. neurologique, 1902, p. 275. 



THE USTTKA-OCULAK MUSCLES 317 

uniform illumination and without the influence of accommodation or 
convergence is known as hippus. It is a clonic spasm of the sphincter 
pupillse (Fick). It may not be associated with impaired vision, but 
is occasionally observed in developing cataract. It is sometimes asso- 
ciated w T ith nystagmus. It is also seen in neurasthenia, hysteria, epi- 
lepsy, disseminated sclerosis, cerebrospinal sclerosis, in acute menin- 
gitis, and in some cases of brain tumor. In observing the changes in 
the size of the pupil, it must be remembered that the normal oscillation 
differs much in different individuals. In patients with irides that react 
very readily to the stimulus of light, oscillation is more pronounced 
than in irides that react slowly. It is difficult to determine where 
normal oscillation leaves off and abnormal oscillation begins. 

According to Schrameck, 55 hippus occurs in cases in which there 
is paresis of the extrinsic ocular muscles, particularly when improve- 
ment is taking place. It has been observed in a case of absence of 
pupillary light reflex (Uhthoff), also in a case of double amaurosis 
(Ewitzky). 

Paradoxical Reflex. — Piltz 56 classifies the paradoxical reflexes as 
follows: (1) Paradoxical reflex (a) on near vision, convergence dila- 
tion of the pupil; (&) contraction of the pupil in distance vision. 
(2) True paradoxical pupillary reflex, (a) dilatation by bright light; 
(5) contraction of the pupil when the light is withdrawn, with sub- 
divisions (aa) rapid dilation without preceding contraction or (bb) 
pupil contracts without preceding dilation. 

Experiments on rabbits and other animals have shown that section 
of the cervical sympathetic on one side causes constriction of the pupil, 
and removal of the superior cervical ganglion on the opposite side 
causes slightly greater constriction of the pupil on the same side, but 
under certain conditions, excitements, anaesthesia, dyspnoea, the pupil 
on the side of the excised superior cervical ganglion becomes more dilated 
than on the side of the divided nerve. This phenomenon has been 
termed paradoxical pupil-dilatation. It has been observed that the 
pupil on the side of the excised superior cervical sympathetic may 
become larger than its fellow even when the . cervical sympathetic on 
the other side has not been divided. 

Lodato 57 writes of the paradoxical pupillary reaction following 
sympathectomy and states that when chloroform is given, the pupil on 



55 Klin. Monatsblt. f. Augenheilk., May, 1902, pp. 45, 46. 

56 Neurol. Centralbl., 20, 22, 1902. 
07 Arch. di. ottal., vol. x„ 3. 4, 1902. 



318 THE EYE AND NERVOUS SYSTEM 

the operated side dilates more considerably than on the non-operated 
side. 

The dilatation must be the result of an increase in the tone of the 
dilatator pupillse muscle due to the excitation, independent of the influ- 
ence of the dilator nerve-fibres in their relation to the superior cervical 
sympathetic ganglion. 

Paradoxical pupillary reflex is rare. It has been observed only 
in patients affected with grave lesions of the central nervous system. 
Bock 58 mentions two cases ; both were tabetic. In one patient there 
were grave lesions of the sympathetic with increased redness and sweat- 
ing of one side of the face. The paradoxical reaction was more marked 
on the side of greater redness and sweating of the face. The vision in 
this eye was greatly reduced. The reaction was obtained from the fel- 
low-eye which possessed % vision but to less degree. The reaction in 
this case was obtained by subjecting the eyes to the influence of bright 
light, dilatation instead of contraction following. 

Accommodative paradoxical pupillary reaction sometimes occurs 
in patients with grave disease of the central nervous system. Piltz 
mentions the rarity of true paradoxical pupillary reaction and cites 
but five cases, all occurring in patients with advanced disease of the 
nervous system. 

Paradoxical pupillary reaction may be confounded with hippus, 
the changes in the pupil which occur in convergence and divergence, 
the effect of heat, and the relaxation of the pupil after forcible con- 
traction of the orbicularis and the production of the so-called lid reflex. 

More than half of the reported cases of paradoxical pupillary re- 
action have been reported as occurring in tabes, 59 but Uhthoff writes 60 
that in all of the vast number of patients observed by him he has never 
seen a true case of paradoxical pupillary reaction. Cases have been 
observed occurring in progressive paralysis, multiple sclerosis, brain 
syphilis. 

The Hemianopic Pupillary Reflex.6i — Lesions of the brain situated 
centrally from the cerebral end of the pupillary light reflex arc, inter- 
fering with vision, do not interfere with the pupillary light reflex. A 
lesion situated in the internal capsule affecting the visual fibres on their 
way to the cortical visual area, or a lesion of the visual area, will pro- 
duce blindness on one or both sides without abolishing the pupillary 

58 Graefe's Archiv, Bd. xvii., H. 2, S. 244. 

69 Muchin Money, Lancet, 1889, p. 173; Abadie and others. 

00 Graefe Saemisch, 2d, Bd. xi., T. ii. p. 240. 

81 Wernicke, Fortschrift d. Med., January 15, 1883, Bd. i., No. 2. 






THE IXTRA-OCULAR MUSCLES 319 

light reflex. This is therefore a valuable symptom in determining the 
location of a lesion affecting the vision. It has been suggested that the 
Haab reflex (page 304) may interfere with the observation of this 
reflex. In regard to this, Haab writes : " In hemianopsia due to cor- 
tical lesions my reflex is not a disturbing factor, as the lesion destroys 
the power of fixing the attention." 

The relation of lesions of the cortical visual areas and the optic 
radiations to the pupillary light reflex may be stated as follows : 

1. Lesion of the cortical visual area on one side gives homonymous 
hemianopsia of the opposite half of the visual fields, Avith fully pre- 
served pupillary light reflex. 

2. Lesion of the cortical visual area on both sides gives total blind- 
ness with fully preserved pupillary light reflex. 

3. Lesion of the tractus on one side and the cortical visual area 
on the other side gives total blindness with pupillary light reflex pre- 
served on the half of the retina corresponding to the side of the affected 
visual area. 

-L Lesion of both tracts causes complete blindness with complete 
loss of pupillary light reflex. 62 

62 For cuts of the pupillary light reflex in hemianopsia, see Plate II 
facing page 290. 



CHAPTER VII. 

PERIPHERAL AFFECTIONS OF THE FIFTH, SEVENTH, AND 
CERVICAL SYMPATHETIC NERVES: OCULAR LESIONS 
CAUSED BY THEM; TREATMENT. 

By EDWARD JACKSON, M. D. 

THE FIFTH NERVE. 

The differentiation between central and peripheral lesions of the 
fifth nerve will often depend on the location of the sensations, or the 
lack of sensation, experienced. Sensations referred to the whole dis- 
tribution of the nerve or to widely separated portions are probably cen- 
tral in origin. Those confined to the region of a single branch are prob- 
ably peripheral. 

Anatomy. — The portions of the fifth nerve having close relations 
with the eye and its appendages are the ophthalmic and superior maxil- 
lary divisions, particularly the former. 

Before leaving the skull the ophthalmic division itself divides into 
lachrymal, frontal, and nasal branches, which enter the orbit through 
the sphenoidal fissure. The lachrymal branch, entering the orbit 
towards the temporal end of the fissure, passes to the region of the lach- 
rymal gland, to which it supplies several filaments ; and is distributed 
to the conjunctiva and portions of the skin indicated in Fig. 1. The 
frontal branch, entering through the upper middle portion of the 
sphenoidal fissure, passes forward near the roof of the orbit to the 
supra-orbital foramen or notch, through which a portion emerges as the 
supra-orbital branch, the supratrochlear having been previously given 
off to emerge from the upper inner angle of the orbit. The nasal branch, 
entering the orbit slightly to the nasal side of the frontal branch, passes 
along the nasal wall of the orbit, enters the ethmoid through the anterior 
ethmoidal foramen, and is distributed chiefly to the nose (See Page 15). 

The superior maxillary division of the nerve, passing through the fora- 
men rotundum across the sphenomaxillary fossa and through the infra- 
orbital canal emerges from the infra-orbital foramen. Its temporal 
and malar branches, given off in the sphenomaxillary fossa, pass through 
the malar bone; and the palpebral and nasal branches, given off after 
it emerges from the foramen, are distributed to the lids, conjunctiva, 
and region of the lachrymal sac. 
320 



THE FIFTH NERVE 



321 



The general distribution of the branches of the fifth nerve to the 
skin of the head and face is shown in Fig. 1. 

The most important anastomoses of the trigeminus are those with the 
sympathetic nerves, especially its connection with the ciliary ganglion. 
It should be borne in mind that beside the superficial distribution indi- 




Supra-trocTilear 
l.V 

Infra-trochlear 
l.V 

Nasal l.V 



Fig. 1.— Normal distribution of the fifth nerve to the face. 1. V— ophthalmic division ; 2. V— su- 
perior maxillary ; 3. V— inferior maxillary. The names on the different areas indicate the branches 
supplying them. (Flower.) 



cated in Fig. 1 the fifth is also the nerve of general sensation for the 
anterior part of the dura mater, and the mucous membrane of the ear, 
nose, throat, accessory sinuses, the mouth, and the upper portion of the 
pharynx. 

Physiology. — Besides serving to transmit the impressions of touch, 
pressure, and temperature, and to excite pain, the fifth nerve is con- 
cerned in very important reflexes. Hyperemia in response to corneal 
irritation may involve the vessels of the optic disk and retina as well 
as those of the pericorneal zone. Contraction of the pupil in response 
to such irritation is often very striking. The increase of the lachrymal 
secretion may be a reflex response to irritation of any portion of the 
mucous membrane supplied by this nerve. Its fibres may also carry 
important efferent impulses governing secretion, or the nutrition of the 
various tissues (trophic). 

Anaesthesia. — In rare cases absence of sensibility over parts or the 
whole of the distribution of the fifth nerve appears to be due to con- 
genital defect. More frequently it arises from lesions due to pressure, 
inflammation, or toxic disturbance of the nerve trunk or its branches. 
Chief among these toxic influences are malaria, lead, and auto-intoxi- 
21 



322 THE EYE AND NEKVOUS SYSTEM 

cation of intestinal origin. The location of the anaesthesia will be deter- 
mined by the nerve distribution. Thus, small areas indicate that the 
course of the disturbance is acting upon correspondingly few peripheral 
filaments. Anaesthesia involving the whole distribution of an important 
branch, such as the supraorbital, indicates that this nerve is affected 
before breaking up into its terminal filaments. When the anaesthetic 
region involves the distribution of all the branches of one of the great 
divisions of the nerve — the ophthalmic or the infraorbital — the cause 
must act close to where such divisions emerge from the skull. The 
anaesthesias of more peripheral origin, and therefore involving smaller 
areas, are very much more likely to be overlooked. This may be the 
reason that the extensive anaesthesias of intracranial origin appear to 
be the more common. 

Corneal anaesthesia is of especial interest. The sensibility of the 
cornea is commonly tested by touching it with a thread or a few 
fibres of absorbent cotton rolled into a point. Eor purposes of com- 
parison, the sound cornea may be similarly tested in alternation with 
the cornea suspected of anaesthesia or hyperesthesia. The sensitiveness 
is to be judged by the apparent reaction produced, as well as by the 
patient's statements. 

The relative ease with which anaesthesia of the cornea is produced 
by the use of cocaine, holocaine, and other local anaesthetics seems to 
indicate that there is an especial liability of the corneal nerves to such 
functional impairment. In malarial disease anaesthesia of the cornea 
may be demonstrated when no other evidence of trigeminal anaesthesia 
can be discovered. The writer has observed it confined to a certain 
segment of the cornea, the sensibility of other parts being normal. The 
corneal anaesthesia of glaucoma is ascribed to pressure on the nerve- 
trunks as they pass forward within the sclera. It is proportioned to 
the increase of the intra-ocular tension, and to the rapidity of that 
increase. 

Paresthesia. — Perverted, abnormal, and usually disagreeable sen- 
sations — like burning, pricking, tingling, numbness, or fulness of the 
parts supplied by the fifth nerve — are more frequently recorded as due 
to central causes. But they may arise from abnormal states, as neuritis 
of the peripheral branches. After injuries — particularly those causing 
bruising of the soft tissues, or fracture of the bones about the orbit — 
such sensations may persist for many months or even for several years. 
Gradually they become less constant and less annoying until they en- 
tirely disappear. After some ocular inflammations, especially inflam- 
mations of the iris and cornea, sensations of the kind may be complained 



THE FIFTH NERVE 323 

of for considerable periods. Some of the discomforts which persist 
after the removal of the eye-strain that had been attended by chronic 
congestion, may be of this character. 

In overcoming these disagreeable sensations, time is the most im- 
portant factor, but something may be done by avoiding, so far as pos- 
sible, any source of irritation or strain, or any observed exciting cause. 

Neuralgia and Neuritis. — Neuralgic pain of peripheral origin is 
probably generally due to neuritis. At least, in practice we are unable to 
distinguish between pain which is due to neuritis and that which is not. 
When such pain is confined to a small area of the skin distribution of 
the fifth nerve, its character may be readily recognized. But when it is 
referred to the eyeball itself, its significance becomes more difficult to 
estimate. The latter will be considered under the next heading. Neu- 
ralgic pain attended with facial spasm (prosopalgia or tic douloureux) 
is discussed under facial spasm. 

Ocular Pain. — It is very important, but often difficult, to deter- 
mine the exact character of pain referred to the eye. The two most 
important varieties are the pain such as is produced by irritation of 
the conjunctival or corneal nerve-endings, and such as would arise from 
deeper lesions. The former is described as scratching, smarting, or 
the feeling of a foreign body ; the latter rather as aching. Burning 
and itching may arise from either set of causes, but these words are 
more commonly used with reference to jDain of superficial origin. 
Boring, shooting, or stinging pains are usually of deep origin, although 
the latter terms may be applied to sensations better referred to as 
scratching. 

The exact location to which pain is referred may be of important 
significance but it cannot be wholly relied on. By the -scratching felt 
in a certain position, the location of a minute foreign body or super- 
ficial lesion may be ascertained. But with regard to pain of deeper 
origin, its referred location is likely to prove misleading. The pain 
of iritis or glaucoma, if severe, will always be felt more or less in the 
brow or cheek. Often it is complained of chiefly or entirely in these 
regions. The mistaking of these diseases for trigeminal neuralgia is 
extremely common among practitioners whose attention is not especially 
fixed upon the eye. Pain referred to the distribution of the ophthalmic 
or superior maxillary divisions of the fifth nerve should always lead 
to a careful examination of the eye-ball ; and pain of ocular origin may 
be even more remotely referred. 

The persistence of para?sthesia after injury has been referred to. 
Pain may be equally persistent. Even though no organic disease may 



324 THE EYE AND NEKVOUS SYSTEM 

be discoverable, pain may continue as a sort of morbid habit. This is 
noticed especially after the relief of eye-strain that has long caused 
headache. But in such cases the suffering gradually diminishes, Some- 
times after an operation on the eyeball or after accidental injury or 
severe inflammation, pain out of all proportion to the severity of other 
symptoms may persist for a long time. In these cases it might reason- 
ably be supposed that a neuritis of the nerve-endings of the fifth nerve 
had arisen ; and treatment directed in accordance with this supposi- 
tion gives, after the necessary lapse of time, satisfactory results. 

Neurotic CEdema. — Localized oedema of the conjunctiva or lids, 
or both, apparently dependent on nerve influence, is not very rare. 
Sometimes it is accompanied by vascular engorgement of the part. But 
in many cases this is not noticeable. The acute attack may be ushered 
in by burning, stinging, or itching of the parts, followed by very rapid 
swelling, which may cause complete closure of the eye. Usually within 
twelve to forty-eight hours the swelling begins to diminish, and in three 
or four days the parts are restored to their normal appearance. Com- 
monly but one eye is affected, but the swelling may extend from one 
side to the other, or both may be originally attacked. Often there is 
similar acute oedema of other parts. But the ocular symptoms may be 
the only ones complained of. 

Some of these attacks closely resemble urticaria in their origin and 
nature. They may be provoked by use of certain articles of diet, as 
fish or shell-fish, or the drinking of alcoholic beverages. In a few 
persons large doses of potassium iodide will produce enormous con- 
junctival swelling of this character. 

Chronic oedema of the lids, liable to increase from errors of diet, 
exposure to wind or dust, use of the eyes, or loss of sleep, is sometimes 
a very annoying and obstinate condition. It may continue to give 
trouble for many years. The treatment includes protection of these 
parts from local irritation , and careful avoidance of the exciting cause, 
when this can be discovered. 

Trophic Disorders. — It is uncertain whether trophic disorders in 
the region of the fifth-nerve distribution are often of extracranial origin. 
But, as their peripheral manifestations are well known and the causa- 
tive lesions unknown or extremely obscure, it is convenient to consider 
them in this connection. 

Facial hemiatrophy involves to some extent the lids and the bones 
of the orbit, but produces no evident alterations in the eyeball itself. 
Unilateral hypertrophy of the face may involve the same parts. Whiten- 
ing or falling of the lashes or hairs of the eyebrow on one side, or a 






THE FIFTH KEKVE 325 

portion of them, is ascribed to morbid nerve influence. Disorders of 
secretion, hyperidrosis, or chromidrosis, localized erythema, itching, 
and perhaps even gangrene, may have a similar origin. 

Neuropathic Keratitis. — Magendie observed that section of the tri- 
geminus in rabbits was followed by inflammation and destruction of 
the cornea. He assumed that this depended upon the loss of a trophic 
influence necessary to sustain the normal corneal nutrition. Snellen 
suggested that lack of sensibility in the cornea permitted injuries to 
occur to it; and foreign bodies to remain in contact with it, by sus- 
pending reflex movements of the lids and lachrymation ; and it was 
shown that after section of the trigeminus, careful protection of the 
eye prevented keratitis. Keratitis can be prevented by fully protecting 
the eye for a few weeks after operative removal or destruction of the 
Gasserian ganglion. Claude Bernard called attention to the share which 
vasomotor changes might have in producing this form of corneal inflam- 
mation. Turner and Ferrier, by experimental divisions of the fifth 
nerve, its roots and branches, with protection of the eye from injuries, 
reached the conclusion that keratitis from injury or disease of the tri- 
geminus was the result of an irritation rather than a paralysis. 

Many recent writers, apparently from an incomplete survey of the 
subject, have accepted the explanation of Snellen, that it was a kera- 
titis of traumatic origin, which might be rendered grave by intercur- 
rent infection. But, as pointed out by Harlan, 1 such an explanation 
does not meet all the known facts. The clinical appearance and course 
of the disease differ radically from those of an ordinary traumatic 
infective keratitis. Harlan reported a case in which the cornea re- 
mained clear under prolonged exposure due to facial paralysis, and sub- 
sequently sloughed when it was protected by paralysis of the elevator of 
the upper lid. In a case reported by Morris the sloughing of the cornea 
occurred in spite of careful protection against injury. It must also 
be borne in mind that neuropathic keratitis occurs in connection with 
trigeminal disease without anaesthesia of the cornea, and that corneal 
anaesthesia due to glaucoma is not attended with any such inflammation. 

In the present state of our knowledge a rational view seems to be, 
that disease or injury involving the trunk or ganglion of the fifth nerve 
causes or predisposes to corneal disease, partly by an obscure irritative 
influence and partly by favoring traumatism and infection. But the 
different hypotheses above mentioned should all be borne in mind, since 
trophic influence, injury, vasomotor disturbances, and central irritation 



Transactions of the American Ophthalmological Society. 1S07. 



326 THE EYE AND NERVOUS SYSTEM 

may all be factors in the case and may require to be met by different 
therapeutic influences. 

The clinical course of the disease may be that of a general soften- 
ing or sloughing of the cornea. More commonly, as the writer has seen 
it, it begins with clouding of the central portion of the cornea. This 
clouding impairs vision. It is accompanied by some general hyper- 
a?mia of the eyeball, but with comparatively little inflammatory pain 
or reaction. Lachrymation is not increased, but the corneal surface is 
not especially dry. The sensibility of the cornea is usually markedly 
diminished. But this may not be the case. Ulceration is liable to 
occur first in the central portion of the cornea. 

The ulcer may become infected, hypopion form, and the cornea 
slough. In a fair proportion of cases the process does not go on to 
perforation; but the cornea heals with permanent opacity. Central 
vision is thus greatly impaired or destroyed, but the eye may still pre- 
serve a useful peripheral field. The corneal disease may be attended 
from an early stage with evidences of iritis, including adhesions, swell- 
ing, and discoloration of the lids. 

Some writers have applied the description of keratitis due to ex- 
posure and desiccation of the cornea — lag ophthalmic keratitis — to this 
disease. The two conditions, however, differ markedly in important 
clinical features. Keratitis from exposure begins with dryness, ulcera- 
tion, and infection in the lower part of the cornea, while in true neu- 
ropathic keratitis it is the central region of the cornea that is first and 
most severely affected. Exposure keratitis has the appearance and runs 
the course of an infective ulcer. Neuropathic keratitis shows a deep 
opacity of the central part of the cornea preceding or without ulcera- 
tion. 

Treatment. — The local treatment of neuropathic keratitis includes 
careful protection of the cornea by union of the lids or the occlusive 
bandage, the cautious application of hot stupes or dry heat, careful 
cleansing of the eye with physiologic salt "solution or very weak anti- 
septic solutions, and the instillation of atropine. The prognosis, how- 
ever, must depend largely upon the nerve element in the case. If this 
can be improved by general regimen, tonics, or the very cautious appli- 
cation of electricity, such measures must not be neglected. 

Herpes Zoster. — Numerous cases of corneal leucoma, associated 
with scars on the face and scalp in the area of distribution of the oph- 
thalmic division of the trigeminus, with the history that this corneal 
disease occurred with an attack of what was regarded as erysipelas, 
called the attention of Jonathan Hutchinson, Sr., to the importance of 






THE FIFTH KEKVE 327 

the eye lesions of herpes zoster affecting this region. An acute clinician 
ought to be able at any stage to recognize that he has to deal with zoster 
rather than erysipelas ; and yet the diagnosis is easier after the case 
has run its course than it is in the early stages. 

Herpes,, unlike erysipelas, is preceded by severe burning, neuralgic 
pain in the affected part, and is not preceded by a chill, high fever, 
marked headache and other evidences of severe general sickness. The 
eruption of herpes zoster begins with isolated spots, upon which vesicles 
develop, instead of having the redness and swelling general from the 
start. After the eruption begins to appear, there is no extension of the 
region affected, and there is but little involvement of the lymphatic 
glands. 

The disease is unilateral, the eruption being sharply limited near, 
but often not exactly at, the median line. The distribution corresponds 
to the more or less variable areas of nerve distribution. The skin of 
the whole affected region becomes inflamed, causing severe swelling of 
the lids and the appearance which gives rise to the diagnosis of ery- 
sipelas. The eruption runs its course in from one to three weeks, each 
vesicle giving rise to a scab which falls off leaving a permanent depres- 
sion. The neuralgic pain may disappear with a full development of 
the eruption, but often it persists, especially in elderly people, after 
all inflammatory symptoms have disappeared , sometimes causing great 
suffering for many weeks or months. 

The ocular lesions attending ophthalmic herpes are quite varied. 
Wilbrand and Saenger have brought together cases of involvement of 
the iris, ciliary body, chorioid, retina, and optic nerve, increase and 
diminution of intra-ocular tension, and paralysis of the internal and 
external muscles of the eye. In a few cases it has been followed by 
paralysis of the facial nerve. These complications appear to present 
the usual characters of inflammation, degeneration, or paralysis of these 
respective parts. 

The common, dangerous, and characteristic ocular lesion is pre- 
sented by the cornea. With the development of the skin eruption vesi- 
cles may appear on the conjunctiva and cornea, giving rise in the latter 
situation to corneal ulceration. The corneal . ulcer may extend and 
perforate. But without perforation and even without ulceration, while 
the eruption is at its height, a dense opacity may develop in the depth 
of the cornea, leaving a permanent leucoma or dense nebula. The cor- 
neal inflammation is apt to last much longer than the skin eruption 
with which it begins; and such clearing of the opacity as does occur 
takes place very slowly, and may not begin until after the persistence 



328 THE EYE AND NEKVOUS SYSTEM 

of the neuralgic pain has ceased to give serious annoyance. The cornea 
will often be found quite anEesthetic, the anaesthesia continuing through 
the period of pain and maximum opacity. In many respects the cor- 
neal disease has the characteristics of neuropathic keratitis. 

Causes. — The pathology of herpes zoster is not fully worked out. 
But the ocular and skin lesions seem to depend upon inflammatory 
changes, hemorrhage, and subsequent degeneration in the Gasserian 
ganglion. The inflammation may extend to the nerve-trunk, and the 
degenerative changes through the roots of the nerve into the central 
nervous .system. Head and Campbell 1 believe that herpes zoster is a 
specific disease with a definite period of incubation, and occurring in 
epidemics. More generally it has been ascribed to cold or to some 
toxic influence. Arsenic and exposure to charcoal fumes have been 
regarded as especially liable to cause it. 

Treatment. — The local treatment should be the same as for neuro- 
pathic keratitis, protective and non-irritative (see page 848). When 
all pain has disappeared and the sensibility of the cornea has been 
largely restored the usual remedies for corneal opacity may be resorted 
to. But the permanent opacity is likely to be out of proportion to the 
loss of corneal tissue. 

Prognosis. — The prognosis varies with the severity of the attack, 
the presence of ulceration, deeper opacity of the cornea, and the occur- 
rence of iritis, or involvement of other deeper structures of the eye. 
In general the danger of a prolonged or permanent disability of the 
eye increases with the age of the patient. It has been noted that the 
cornea is more likely to suffer in those cases in which vesicles appear 
on the side of the nose. But severe corneal lesions may occur when 
the skin of the nose is not at all involved, and the cornea may escape, 
although well-marked vesicles or scars are found upon the nose. In 
all cases the prognosis should be guarded, especially as to the time of 
recovery. Some eyes remain irritable and painful many months and 
years after the attack, and there is no way of judging in the early stages 
which cases will run such a course. 

Other Forms of Herpes. — It must not be forgotten that other forms 
of herpes occur in and about the eye. In rare cases the ordinary febrile 
herpes has been observed occurring on the lids as it generally occurs 
upon the lips. Herpes of the cornea occurs in connection with various 
febrile diseases, especially affecting the respiratory tract. Symptoms 
of severe corneal irritation attend the development of the vesicles, and 

1 Brain, 1900, No. 91. 



THE FIFTH aSTEKVE 329 

their rupture leaves a number of superficial ulcers. But these ulcers 
commonly heal without leaving any permanent impairment of vision. 
Phlyctenular disease of the conjunctiva and cornea also exhibits many 
of the characteristics of an herpetic eruption. There can be little doubt 
that the peripheral branches of the fifth nerve perform an essential 
part in the production of all these lesions. In febrile herpes the dis- 
turbance may be due to poisoning or impaired nutrition of the nerve. 
In phlyctenular disease it has been ascribed to the local action of various 
bacterial toxines. 

Recurring Erosion of the Cornea. — After a superficial injury, as a 
finger-nail scratch of the cornea, attacks of severe pain with or without 
loss of epithelium may recur for months or even years. Injury to the 
peripheral nerve-endings of the cornea is probably an essential factor in 
producing this condition. 

Dendritic ulcer of the cornea, as has been pointed out by Charles, 1 
probably owes its peculiar character to lesions affecting the terminal 
filaments of the corneal nerves between Bowman's membrane and the 
epithelium. 

Supposed Reflex Blindness and Palsies. — From the time of Hip- 
pocrates it has been known that various injuries in the region supplied 
by the fifth nerve were liable to be followed by blindness or by paralysis 
of one or more of the ocular muscles. It is now known that in many 
of these cases the blindness or paralysis is due to direct injury to the 
optic nerve or to the motor nerve supplying the affected muscles. 
Wounds about the orbit often penetrate more deeply than is suspected, 
and hemorrhage or thrombosis may carry their effects still deeper. 
Post-mortem examination has demonstrated that of fatal cases of frac- 
ture of the cranium from blows upon the region supplied by the fifth 
nerve the fracture involved the orbit in 68 per cent., and in 51 per 
cent, extended into the optic foramen. 

Nevertheless, the view that optic nerve atrophy and paralysis of 
the motor nerves of the eye might be produced by trophic influences 
set up by injury to the peripheral branches of the fifth nerve has been 
accepted by many excellent observers, even in recent years. Hubbell, 2 
who has recently supported this hypothesis, cites a long list of famous 
clinical observers who are inclined to accept it. To the writer it seems 
more reasonable to suppose that all cases of the class are caused in the 
same or closely similar manner in which we know that a large propor- 



American Journal of Ophthalmology, April, 1904. 

Transactions of the Section on Ophthalmology of the A. M. A., 1904. 



330 THE EYE AND NERVOUS SYSTEM 

tion are caused, than to have recourse to an hypothesis so indefinite and 
unsupported as that of a harmful trophic influence exerted by the fifth 
nerve. 

Photophobia.— Although the term photophobia (fear of light) 
might be expected to indicate only symptoms arising from the retina 
and optic nerve, it is applied to phenomena more generally connected 
with irritations acting upon the peripheral branches of the fifth nerve. 
It is easy to see how light falling upon an eye with an inflamed iris 
may cause disturbance of the nerves of general sensation distributed 
to the iris by the reaction it provokes causing movements in the inflamed 
tissue. But in some of the most marked cases of photophobia it is 
evident that light has little or nothing to do with the production 
of the symptoms. The child with phlyctenular keratitis buries its 
head in the pillow as strenuously and persistently at night or in the 
darkened room as in full daylight. And its fear of light is removed 
by measures that diminish the hypersensitiveness of the fifth-nerve 
endings, but which have no appreciable effect upon the retina and optic 
nerve. 

There is a form of discomfort about the eyes, and aching often 
becoming a general headache, which seems directly connected with 
exposure to light. This may be experienced by most persons who are 
much indoors with feeble illumination. When they go directly into 
strong sunlight, the pain may arise instantly, and may have the char- 
acter of a sharp cramp, probably connected with excessive contraction 
of the sphincter of the pupil. The contrast between bright lights and 
comparative darkness, produced by looking towards sources of artificial 
illumination at night, is quite unpleasant to most people. Excessive 
liability to similar unpleasant sensations is noted in patients suffering 
from acute or chronic wasting disease, attended with toxaemia, especially 
after typhoid fever and in the course of general tuberculosis. Photo- 
phobia of the kinds above mentioned may have little connection with 
the fifth nerve, and that only through the nerve-centres. 

But the photophobia which attends the presence of a foreign body 
in the eye, or inflammatory irritation of the conjunctiva, cornea or iris, 
is clearly a fifth-nerve symptom. It is associated with no characteristic 
anatomical changes in the nerve. Indeed, the production of this form 
of pain can hardly be regarded as an abnormality of the nerve function, 
since it seems necessary to secure rest and protection or relief from 
the irritation that causes the photophobia. In this form of photophobia 
there is a tendency to spasmodic closure of the lids, excessive lachry- 
mation, and general hyperemia of the eyeball. In the more severe 



THE FIFTH NERVE 331 

•cases the face may be continuously buried, in a pillow, cushion, or hand- 
kerchief, or the eyes exposed to the air and light only with great diffi- 
culty, and for a short time. 

Treatment. — Photophobia which is rarely connected with exposure 
to light should be met by measures that will lessen the general erethism 
of the nervous system, and by avoiding contrasts of illumination, either 
.successive, by going from darkness suddenly to light, or simultaneous, 
.as by facing sources of artificial light at night, or the narrow streaks 
of light about a shaded window. But it is important not to have the 
patient give way too much to the desire to avoid diffuse daylight. If 
dark glasses are worn they should be resorted to only at times of ex- 
posure to the brightest light, so as to lessen the contrast. They should 
not be used continuously. Habitual exposure to moderate light is the 
physiologic and most efficient method of keeping down the hypersensi- 
tiveness of the retina. 

Photophobia dependent on corneal irritation demands chiefly the 
removal of the irritation. The avoidance of exposure to light and air 
may, however, tend to increase the photophobia. Often the dressings 
worn upon the eye aggravate and continue the symptom when it would 
•otherwise have subsided. Photophobia is usually an indication for 
removal of bandages and avoidance of anything that will keep up the 
heat and moisture of the parts. It is often wise to substitute dark 
glasses for a close dressing, simply because they permit the free access of 
air, and continuous cooling of the parts by evaporation. Strongly 
-astringent applications, as silver nitrate, boroglyceride, etc., do good 
by removing the hypersensitiveness of the nerve-endings. 

In photophobia attended by lachrymation small fissures in the skin 
:are apt to appear about the outer canthus (rhagades). The treatment 
of these, as by touching with a strong solution of silver nitrate, will 
greatly lessen the photophobia. The local application of cocaine or 
holocaine within the conjunctival sac temporarily relieves photophobia. 
But with the passing of the anaesthetic influence the symptom returns 
with renewed force, and frequent reapplications of cocaine to keep 
up the anaesthetic influence are extremely dangerous. 

In corneal disease the influence of atropine upon the nerve-endings 
in the cornea gives a decided and much more permanent relief, and 
usually proves beneficial also for the condition causing the photophobia. 
"Where photophobia is attended with spasmodic closure of the lids and 
does not yield to other measures, it will usually be greatly lessened by 
canthotomy or canthoplasty. The old plan of suddenly plunging the 
head into cold water is efficient, but not often practical. 



332 



THE EYE AKD KEKVOUS SYSTEM 



THE SEVENTH NERVE. 

The course of the seventh, or facial, nerve through the temporal 
bone, and its numerous anastomoses and chief branches of distribution, 
are shown in Fig. 2 (Compare Page 17). 

Entering the internal auditory meatus it lies in close relation with 
the auditory nerve, so that any gross lesion of this portion will be 
attended by vertigo, abnormal movements, and deafness of the same 
side. But there is no deafness or hemiplegia of the opposite side, 



ggl-spheno pa 




Fig. 2.— Seventh nerve (facial) and its branches and anastomoses. (Leube.) 



or abducens paralysis of the same side, which would attend an intra- 
cranial lesion. Leaving the auditory nerve, it enters the Fallopian 
canal. At the geniculate ganglion, where the nerve and canal bend 
sharply downward, are given off the petrosal branches, communicating 
with the spheno-palatine ganglion and the otic ganglion. Clinical 
evidence of the involvement of these branches has not been recognized. 
A little lower a branch is given off to the stapedius muscle, and 
paralysis of this muscle may cause abnormal acuteness of hearing. 
Hence a lesion occurring in the Fallopian canal, but not involving its 
bony wall or the structures contiguous to them may be attended by 
hyperacusis instead of by deafness. A little lower still the chorda 
tympani is given off. Lesions centered at this point are attended with 
disturbance of the salivary secretion and taste. More peripheral lesions 
leave taste undisturbed, while affecting all the external muscles inner- 
vated by the facial nerve. At the stylomastoid foramen the posterior 
auricular, digastric, and stylohyoid branches are given off, so that extra- 



THE SEVENTH KEKVE 



333 



cranial lesions leave them, unaffected. There are also minnte twigs 
sent to the sympathetic and glossopharyngeal and pneumogastric nerves. 

Emerging from the stylomastoid foramen, the facial nerve runs 
within the parotid gland across the external carotid artery. Behind 
the ramus of the lower jaw it divides into the cervicofacial branch, 
distributed to the neck and lower part of the face, and the temporo- 
facial, which splits up into the temporal, malar, and infra-orbital 
branches. The temporal and malar branches supply the oculofacial 
muscles ; that is, the frontal portion of the occipitofrontalis, the orbicu- 
laris palpebrarum, and the corrugator supercilii. 

Paralysis of the Seventh Nerve. — Eacial paralysis of interosseous 
or external origin (Bell's palsy) is attended with loss of faradic irri- 
tability in the affected muscles which occurs in the first week, and 





Fig. 3.— Peripheral facial paralysis, right side. 
(Case of Dr. Spiller.) 



Fig. 4.— Bilateral peripheral facial pa- 
ralysis ; attempt to close both eyes and 
draw up the corners of the mouth. (Case 
of Dr. Spiller.) 



later with wasting of the muscles. The loss of function is shown by a 
general relaxation of all the muscles of one side of the face, as illus- 
trated in Fig. 3. The " smoothing out" of the face produced by this 
relaxation varies with the amount of subcutaneous fat and the habitual 
wrinkling of the skin. It is less noticeable in women and children, 
more pronounced in the aged and those Avith deeply furrowed faces. 

There is also inability to execute the usual movements of the face, 
as to elevate the brow or close the eye (Fig. 4). It is generally 
advised to test the power of closing the eyes by having the patient attempt 
to close each eye separately. But this may prove misleading, for some 
persons cannot close either eye separately and others can close one eye 



334 



THE EYE AND KEKVOUS SYSTEM 



alone but not the other. The latter is commonly the case where one eye 
has distinctly better vision than its fellow. It is better to have the patient 
try to close both eyes at once or to elevate both brows as nearly alike as 
possible. One may also detect the lessened power of the muscles by 
offering resistance to their action, as by resisting closure of the lids. 
This may be made a rough test of recovery. 

While in paralysis of central origin the oculofacial muscles are 
usually but slightly involved or may quite escape, it is possible to have 
a peripheral paralysis confined to these muscles. Thus Mills mentions 
a case in which from a stab in front of the ear the frontalis was para- 





Fig. 5.— Contracture of the orbicularis 
palpebrarum and of other muscles of the 
right side of the face in facial paralysis of 
central origin. (Case of Dr. Spiller.) 



Fig. 6.— Total peripheral facial paralysis 
of right side. Secondary contracture. In- 
voluntary closure of eye and elevation of 
brow in laughing. (Wilbrand and Saenger.) 



lyzed and the orbicularis palpebrarum was weakened, although the other 
facial muscles were unaffected. In another case, following a fall on 
the temple, the loss of power was confined to the frontalis (page 439). 
Paralysis of the frontalis destroys the power of elevating the eye- 
brow. This leaves the brow drooping on the affected side, narrowing 
somewhat the upper part of the field of vision. Paralysis of the orbicu- 
laris prevents closure of the lids. The consequences of exposure of 
the eye will be discussed under lagophthalmos. It also permits the lower 
lid to hang away from the eyeball, interfering with the lachrymal 
function. (See Laciirymation.) After three or four months secondary 
contractures may occur in the paralyzed muscles. These are less fre- 
quently exhibited in the oculofacial muscles than in those acting on 
the mouth. But they may obscure the diagnosis, and even give the 
impression that the paralysis affects the opposite side ' (Pig. 5). 



THE SEVENTH NERVE 335 

The contracture of the orbicularis causes narrowing of the palpebral 
opening and wrinkling of the neighboring skin. The contracture of the 
frontalis causes an undue elevation of the eyebrow of the affected side. 
They are increased during voluntary action of the affected muscles, as 
in laughing. The effects of such contracture are well shown in Eig. 6. 

Etiology. — A large proportion of cases give a clear history of recent 
special exposure to cold, which seems to act by setting up a neuritis 
or perineuritis. Less frequently the neuritis seems to be of toxic origin. 
Syphilis, rheumatism, diabetes, gout, leukemia, diphtheria, and other 
specific infections are credited with causing it. It has also been 
ascribed to an acute specific infectious process. A considerable num- 
ber of cases are secondary to aural disease, particularly suppuration 
of the middle ear, and local injury to the nerve or its branches may 
cause it. It may occur as a part of a polyneuritis. Shumway 1 has 
collected eight cases in which it has been accompanied by optic neuritis 
and subsequent optic atrophy. In two cases he has seen it followed 
by marked flattening of the face on the affected side and ptosis. 

Treatment. — This must first combat the cause. In neuritis due to 
cold it is important that active treatment should begin promptly. A 
general purgative, leeches below the ear, salicylates and later iodides 
are to be tried. Counterirritation by a blister or by milder irritants 
may prove useful. At a later stage strychnia may be given. Elec- 
tricity is to be used with caution, and its application immediately sus- 
pended if there arises evidence of secondary contracture (page 885). 

Prognosis. — The j>rognosis depends upon the cause and, with neuritis 
due to cold, somewhat on the promptness with which effective treatment 
is begun. Probably half of all cases make a fair recovery, but a con- 
siderable proportion show permanent loss of power in the affected mus- 
cles, and in not a few the paralysis continues complete. The rapid and 
complete loss of faradic irritability is unfavorable, and complete re- 
covery cannot be expected where secondary contractures have occurred. 
Long duration of the paralysis is also a ground for an unfavorable 
prognosis, although cases may recover after several months. 

Facial Spasm. — This is more frequently of cerebral or nuclear than 
of peripheral origin. But it may be caused by a gross irritative lesion 
in any part of the nerve-trunk or its branches. Meige 2 classes as 
" spasms" those motor reactions arising from actual irritation, and as 
" tics" those disorders which have a psychomotor origin, and which are 



1 Trans. Sec. on Ophthalmology., A. M. A.. 1004. 

2 Annates d'Oculistique, March. 1003. 



336 



THE EYE AND NEKVOUS SYSTEM 



therefore not discussed in this chapter. Certain forms of spasm are 
discussed in connection with the palpebral opening. We are here con- 
cerned with spasm of the frontalis, the corrugator supercilii and the 
orbicularis palpebrarum muscles, and only the last, blepharospasm, is 
of much practical importance. 

Blepharospasm. — Blepharospasm varies from slight fibrillary 
twitchings of the orbicularis muscle, to painful contraction of the mus- 
cle, which resists strong mechanical effort to separate the lids, and 
entails periods of practical blindness. The fibrillary twitchings may 
be observed shortly after the instillation of eserine. Many persons 
experience them after loss of sleep or moderate overexertion of the 



«, •*■" 



Fig. 7.— ( 



of tic douloureux: appearance of patient's face during severe paroxysm. (Mills). 



eyes. They are often quite annoying to the patient, although scarcely 
perceptible to anyone else. They have no grave significance, and can 
be relieved by sleep, removal of any local irritation or of eye-strain. 
But they are liable to recur in persons predisposed to them. 

Excessively frequent, somewhat prolonged acts of winking are often 
noticed in children. They call for careful search for some local irri- 
tation or eye-strain. Older persons, who have usually suffered from 
prolonged conjunctival irritation, acquire a habit of exaggerated wink- 



THE SEVENTH KEKVE 337 

ing, a rather prolonged, forcible closure of the lids. This habit be- 
comes a serious complication when certain operations, especially cataract 
extraction, are required. 

More acute and often uncontrollable blepharospasm is a symptom 
of conjunctival, and especially of corneal, irritation. It is usually asso- 
ciated with photophobia and excessive lachrymation. This is particu- 
larly marked as a symptom of phlyctenular disease. The pulling of a 
tooth will cause a sudden reflex spasm of all the muscles of that side 
of the face. Forcible uncontrollable spasm of the lids occurs in tic 
douloureux or prosopalgia (Fig. 7). Hysterical blepharospasm is dis- 
cussed in the chapter on hysteria (page 651). In a few cases attacks of 
spasmodic closure of the eyes without discoverable cause occur with grad- 
ually increasing frequency and prolongation of the attacks. Mills sug- 
gests that these are due to nuclear instability, — a manifestation of 
chronic degenerative changes. 

Diagnosis. — All forms of blepharospasm tend to cause narrowing 
or closure of the palpebral opening, and wrinkling of the skin of the 
lids and adjoining parts. Ptosis causes narrowing of the palpebral 
opening, but is rather attended with smoothness of the lids from re- 
laxation or distention of the parts. But, besides recognizing the con- 
dition present as blepharospasm, the diagnosis must include the 
determination of its cause, if possible. Careful search should be made 
for a source of irritation, as inflammation or the presence of a foreign 
body. Beginning with the cornea and conjunctiva this search must 
extend to the regions supplied by other branches of the fifth nerve, 
including investigation of possible disease of the maxillary, nasal, and 
other adjoining sinuses, and of the teeth. 

Treatment. — The treatment of blepharospasm is first, and usually 
wholly, the removal of its cause. This must often include the correction 
of ametropia or other possible cause of eye-strain, as well as the re- 
moval of mechanical or inflammatory irritation. In a few chronic cases 
in which the spasm causes severe pain or temporary disability by blind- 
ness, it is right to resort to stretching, division, or excision of the nerve- 
branches concerned. These measures will give relief for the time, some- 
times for many months, but their effect is not often permanent (p. 894). 

Prognosis. — This is favorable in proportion as the spasm is due to 
an evident and remediable irritation. Cases not so caused, and espe- 
cially those due to chronic degenerative changes, are well-nigh hopeless. 
Certain tics of central or psychomotor origin have a better prognosis, 
being amenable to educative treatment. 

Spasmodic Entropion. — The tendency to exaggerated winking pro- 
22 



338 THE EYE AND NEKVOUS SYSTEM 

duced by any irritation in the conjunctival sac, with the chronic inflam- 
matory changes that produce it, are the causes of spasmodic entropion. 
Entropion caused by relaxation or swelling of the skin of the lids is 
often classed with this. But the true spasmodic form depends on the 
action of the orbicularis. When this muscle is strongly contracted, the 
palpebral opening is narrowed, and the skin around is dragged in 
towards it. When this dragging is kept up, or repeated at short inter- 
vals over a considerable length of time, the skin at the lid margin 
becomes more or less pushed over the edge of the cartilage toward the 
inner side of the lid, carrying with it the lashes, which then rub against 
the eyeball, and still further increase the irritation and tendency to 
spasmodic contraction of the orbicularis. Entropion most frequently 
occurs in the course of trachoma, in which there are, with conjunctival 
irritation, cicatricial changes in the lids leading to incurving of the 
tarsus and organic entropion. In these cases it is hard to separate the 
spasmodic from the cicatricial causes. But the orbicularis spasm may 
be an important factor in the development of organic entropion. 

Treatment. — This must include, first, the removal of the irritation 
which is provoking the orbicularis to spasmodic contraction, as the 
treatment of the trachoma. In some cases the habit of spasmodic 
closure of the lids is itself an important cause of irritation, and can- 
thotomy or canthoplasty, preventing the excessive pressure of the lids 
upon the eyeball, will be followed by great improvement. In severe 
chronic cases, however, an entropion operation is indicated, especially 
that of Hotz, which fastens the skin of the lid securely to the upper 
margin of the tarsus. 

THE SYMPATHETIC NERVE. 

Anatomy. — The sympathetic nerve supply of the eye and its ap- 
pendages comes through the superior cervical ganglion of the sympa- 
thetic, which Langley calls " a relay station for the sympathetic nerve 
supply for the whole of the head." Erom this ganglion branches form 
plexuses which accompany all the arteries distributed to the orbit and 
surrounding regions, and other branches, mostly from the larger plex- 
uses, join the cranial nerves. The third receives such a branch at its 
point of division; the fourth as it lies in the wall of the cavernous 
sinus; the fifth in the Gasserian ganglion; the sixth where it lies in 
close apposition with the carotid plexus; the seventh at the stylo- 
mastoid foramen. 

In the orbit is situated the ciliary ganglion. This is about 2 milli- 



THE SYMPATHETIC NERVE 339 

metres long and 1 millimetre thick. It lies slightly to the temporal 
side, and slightly lower than the optic nerve, 8 to 11 millimetres from 
the optic foramen, and 14 to 17 millimetres from the entrance of the 
optic nerve through the sclera. Its roots are derived from the fifth 
nerve, usually from a branch of the nasal just after entering the 
orbit, a filament of the carotid plexus, and a filament from the oculo- 
motor, usually from the branch supplying the inferior oblique. It 
gives off the short ciliary nerves, which enter the posterior part of the 
sclera, and run forward between it and the chorioid to the ciliary 
muscle and iris. We thus have the sympathetic reaching all parts of 
the eye and its appendages, and a special mechanism supplying the 
iris and ciliary body, the function of which is discussed in Chapter VII. 
on " The Intra-ocular Muscles." 

Vasomotor Disturbances. — The most general and best understood 
function of the sympathetic nerve is its vasomotor function. Uni- 
lateral flushing of the face and neck occurs, and may be attended with 
noticeable hypersemia of the conjunctiva and narrowing of the pal- 
pebral opening. Such an attack may last but a few minutes or it may 
continue for hours. A more permanent impairment of this function 
may occur. Following influenza, the writer has seen hypersemia of 
one eye, especially of the conjunctiva, with narrowing of the palpebral 
opening and slight tonic ptosis. Cases of this kind have not received 
the attention they deserve. It is very easy to confuse such a condition 
with unilateral inflammation of the conjunctiva and partial closure of 
the lids on account of irritation. But instead of active local treatment 
these are to be benefited chiefly by improving the general condition. 
Pulsating exophthalmos not due to gross lesions of the orbital vessels 
may be regarded as a vasomotor disorder. 

The connection of the general blood-pressure with the intra-ocular 
tension has been the subject of various theoretic discussions and physio- 
logic experiments. The conclusions drawn from the latest of these by 
Parsons and Snowball 1 are that the intra-ocular tension responds pas- 
sively to all variations in the general blood-pressure, whether these be 
produced by vasomotor actions or by such mechanical means as the tying 
of a large artery or by the inhibition of the heart's action. The in- 
crease of intra-ocular tension caused by stimulation of the peripheral 
portion of the cervical sympathetic they ascribe, however, not to vaso- 
motor action but to contraction of the unstriped muscles of the orbit. 

Influence of Unstriated Muscles. — Stimulation of the sympathetic 



Royal London Ophthal. Hosp. Reports, vol. xv. Part 3, p. 275. 



340 THE EYE AND NERVOUS SYSTEM 

produces contraction of the unstriated muscle-cells with which it is 
connected. The intra-ocular muscles and their action are discussed in 
the preceding chapter. The principal extra-ocular muscles of this char- 
acter are those acting upon the upper and lower lids; and the fibres 
stretched across the sphenoidal and sphenomaxillary fissures. These 
latter are better developed in some of the lower animals; and it is 
doubtful if they have much practical importance in the human orbit. 

Contraction of Mueller's muscles connected with the lids tends to 
cause retraction of the lids. Their relaxation produces narrowing of 
the palpebral opening. Contraction of fibres stretching across the back 
of the orbit has been suggested as the explanation of the protrusion 
of the eyes produced by stimulation of the sympathetic, and relaxation 
of these fibres has been supposed to account for exophthalmos. But it 
is doubtful whether the protrusion and sinking, produced by stimula- 
tion or paralysis of the sympathetic in man, are not merely apparent 
rather than real, the appearance being produced by the widening or 
narrowing of the palpebral opening. The detailed effects produced 
by these muscles are discussed in connection with alterations of the 
lids and the palpebral opening. 

Secretion. — One-sided sweating with flushing of the face occurs 
from defective sympathetic innervation. In these cases the eyelids 
show slight increase of secretion. The influence of the sympathetic 
upon the lachrymal secretion is discussed under that heading. What 
share its influence on secretion has in affecting the intra-ocular tension 
will be considered under the next heading. 

Intra-ocular Tension. — Softening of the eye-ball after section of 
the cervical sympathetic was one of the earliest observations regarding 
the physiology of this nerve. Increase of intra-ocular tension by stimu- 
lation of the peripheral portion of the nerve is another common obser- 
vation. How these changes of intra-ocular tension are produced is still 
in dispute. Hippel and Gruenhagen ascribed them to influences pro- 
duced upon the orbital muscles and the vessels of the eye. Adamiik 
attributed them to changes in the intra-ocular muscles, especially to the 
unstriped fibres of the choroid. Horner and Nicati concluded that the 
change was independent of the ocular vessels. Abadie suggested that 
excitation of vasodilator fibres was the cause of increased tension. 
Lodato causing, by insertion of a foreign body, irritation of the superior 
cervical ganglion, produced elevated intra-ocular tension lasting for some 
months. But Weeks, among reported cases of irritation of the cervical 
sympathetic in man, found no mention of glaucoma occurring as a 
complication. Selenkowski and Rosenberg, Hertel and Levinsohn found 



THE SYMPATHETIC NEBVE 341 

the changes of tension produced by disturbance of the cervical sympa- 
thetic were but brief. De Schweinitz * concludes that the fall of the intra 
ocular tension produced by section of the sympathetic or extirpation of 
the sympathetic ganglion probably depends on vascular or perhaps mus- 
cular changes. 

It is quite possible that the sympathetic exerts, apart from its vaso- 
motor function, a direct control over secretion within the eye. But 
no one has yet been ingenious enough to devise appropriate experiments 
or to so marshal clinical facts as to clearly demonstrate this 
condition. 

Within the last ten years excision of the superior cervical ganglion 
of the sympathetic has been done many times for exophthalmic goitre, 
epilepsy, and glaucoma. From the clinical observations on these cases 
and those in which the sympathetic has been paralyzed, as by pressure 
from enlarged cervical glands, it seems clear that loss of function of 
the cervical sympathetic is followed by diminished intra-ocular tension. 
In healthy eyes this diminution seems to be brief and unimportant. 
In glaucomatous eyes it may be more prolonged. 

Wilder 2 concludes that " sympathectomy for glaucoma is not an 
operation to be condemned too hastily. . . . The statistics up to date 
seem to indicate that the simple chronic form is the one most suited 
for it, next to the hemorrhagic form, if that can be determined. '' 
What is yet known with reference to the influence of the sympathetic 
upon intra-ocular tension does not justify neglect of older established 
methods of' controlling this condition. But it does justify resort to 
sympathectomy as an adjunct to other treatment or when such treat- 
ment has proven insufficient. 

Excision of the ciliary ganglion practised by Bohmer 3 is a difficult 
operation on account of the smallness and position of the ganglion; 
and it has not proven so efficient as excision of the superior cervical 
ganglion for the reduction of intra-ocular tension. It should be con- 
sidered not as an alternative of iridectomy or even of sympathectomy, 
but rather in comparison with opticociliary neurotomy or excision of 
the eyeball. 

Pathology. — While the effects of increased or diminished func- 
tional activity in the sympathetic nerve are well understood, the re- 
corded observations as to its pathological histology are so inconclusive 
as to furnish no basis for a classification of its diseases. Indeed, they 

1 Trans. Sec. on Ophthalmology, A. M. A., 1903. 

2 Ibid. 

3 Annates d'Oculistique, July, 1902. 



342 THE EYE AND NERVOUS SYSTEM 

kere require but little notice. Weeks 4 finds regarding glaucoma : 
" The testimony in our possession is not sufficiently conclusive to enable 
us to say that there is any constant change in the cervical sympathetic 
peculiar to glaucoma. Nor is it sufficiently conclusive to exclude the 
possibility of such constant change." The most constant change found 
in the ganglia removed has been an increase of pigment in the neuron 
cell-bodies. But this may be a senile change. Increased connective 
tissue, or sclerosis, which has been reported by several observers, has 
not been found at all by others equally competent. 

THE SECRETION AND REMOVAL OF TEARS. 

Secretion. — The control of nerve impulses, either of reflex or of 
central psychic origin, over the secretion of tears is one of the com- 
monest observations in ocular physiology. It is also certain that reflex 
lachrymation is especially likely to arise from impressions made through 
the fifth nerve, as by the irritation of wind or dust in the eye, pungent 
gases, etc. But the exact channels by which the efferent nerve impulses 
governing its action reach the lachrymal gland have not been determined. 

While fibres of the sympathetic nerve enter the gland along each 
of its nutrient vessels, its most evident nerve-supply is through branches 
of the fifth nerve. Chiefly it is supplied through the lachrymal branch 
of the ophthalmic division, which runs forward near the roof of the 
orbit to be distributed to the gland. But anastomosing with this is a 
twig coming from the orbital branch of the second division of the tri- 
geminus ; and this anastomotic twig may have special importance with 
reference to the lachrymal secretion. A large part of the lachrymal 
branch of the first division passes through the gland to be distributed 
to the conjunctiva and skin adjoining. (See Fig. 8.) 

The results of physiologic experiments are so contradictory, pos- 
sibly on account of the variations of function of the different animals 
experimented upon, that they throw no certain light upon the neurology 
of the lachrymal secretion. They need not be here discussed, except 
to point out that increased secretion of tears after section of the sym- 
pathetic or after removal of the superior cervical ganglion is usually 
quite temporary, and probably depends upon vasomotor paralysis. 

When it comes to actual disease of the nerves, lesions of the fifth 
nerve seem less frequently to cause disturbance of lachrymation than 
those of the seventh. In trigeminal neuralgia there is no constant or 
characteristic alteration of the lachrymal secretion. Nor is any lach- 



4 Trans. Sec. on Ophthalmology, A. M. A., 1903. 



SECKETION AND KEMOVAL OF TEAKS 



343 



rymal disturbance more certain to occur, with neuralgia involving the 
first division of the nerve, than when the second or third division is 
affected, without the first. The intense pain may cause the increased 
lachrymation, very much as pain elsewhere, or irritation of the eyes 
or nasal mucous membrane may cause it. A more specific disturbance 
of the lachrymal function has, however, been noticed by UhthofT in 
one case of peripheral neuritis affecting the first and second divisions 
of the fifth nerve. 

Paralysis of the seventh nerve, by relaxation of the orbicularis, 
allowing the lid to fall away from the eye-ball, commonly disturbs 




Fig. 8.— Nerve supply of lachrymal gland. 1, lachrymal branch ; 2, lachrymal gland ; 3, malar 
branch ; 4, second division of fifth nerve ; 5, anastomotic branch connecting gland with second division. 
(Merkel.) 

the removal of tears. But in a considerable number of reported cases, 
facial paralysis has clearly been attended with diminution, if not com- 
plete cessation, of the secretion of tears on the affected side. Probably 
the symptom would be more frequently noticed if the diminished re- 
moval of tears did not mask the diminished secretion. The interference 
with secretion, and the interference with removal, to some extent neu- 
tralize each other ; and render the lachrymal disturbances attending the 
seventh nerve lesions less noticeable than they otherwise would be. 

While the neurology of lachrymation cannot be regarded as settled, 
the share of the seventh nerve in the control of the gland might be 



344 THE EYE AND NERVOUS SYSTEM 

explained in this way: As has already been pointed out (page 333), 
the seventh nerve gives off at the geniculate ganglion the petrosal 
branches ; the superior, or greater, petrosal passes to the sphenopalatine 
ganglion, which gives branches to the second division of the trigeminus ; 
and this, as we have seen, supplies an anastomotic branch to the lach- 
rymal nerve. Through this channel efferent impulses, emerging from 
the nerve-centres by way of the seventh nerve, may reach the lachrymal 
gland. The similarity of the arrangement with that of the chorda 
tympani, given off from the seventh more peripherally than the great 
petrosal, and joining the third division of the trigeminus, is quite 
striking. That the chorda tympani exerts an important influence over 
the secretion of the salivary glands is well known, and this view of 
the nerve-control of the lachrymal secretion adds one to the striking 
analogies that may be traced between the functions of salivation and 
lachrymation. It should not be forgotten, however, that definite proof 
regarding the innervation of the lachrymal gland is yet lacking. 

The amount of lachrymal secretion formed in a given time depends 
almost wholly upon reflex influences. Thus, Schirmer points out that 
the secretion practically ceases during sleep. Under psychic influences 
or peripheral irritation, it is capable of enormous increase. Even in 
those persons in whom epiphora is most constant it occurs in response 
to some especial stimulation, as from chronic inflammation of the lach- 
rymal sac, eye-strain, or the ordinary exposures, which in the particular 
patient cause an excessive reflex. It is a significant fact that after 
removal of the lachrymal sac for dacryocystitis, the patients usually 
have no further trouble from epiphora, showing that it has been a reflex 
of the irritation of the diseased tear passages. 

Increased or diminished lachrymation may occur in organic disease 
of the central nervous system, as locomotor ataxia. In exophthalmic 
goitre increased lachrymation has usually been ascribed to exposure of 
the conjunctiva by retraction of the lids and defective winking. But 
Berger has pointed out that it may antedate the symptoms of Dal- 
rymple and Stellwag (See Pages 668 and 840). 

Increased or diminished lachrymation may occur in organic disease 
Schirmer, from his very careful study of the subject, concludes that 
in the act of winking, tension upon the palpebral tendon causes a dila- 
tation of the lachrymal sac, and the sucking into it of tears from the 
conjunctiva. When the eye is reopened the elasticity of the sac forces 
the tears on into the nose. Probably an important part in the process 
is taken by the minute sphincter muscles which surround each lachry- 
mal punctum. Relaxation of these sphincters during winking favors 



THE LIDS AND THEIK MOVEMENTS 



345 



the passage of the tears into the canaliculi; and their contraction at 
other times tends to prevent regurgitation. 

The abnormal, continuous relaxation of this little sphincter muscle, 
either by chronic disease or forcible dilatation, impairs the natural ability 
to remove any excess of lachrymal secretion. In order that the tears 
shall enter the canaliculi the puncta must be turned in towards the 
eyeball, and the lids be in contact with the globe of the eye, so that 
the tears shall be conducted by capillary attraction to the puncta. 
Paralysis of the orbicularis causes interference with the passage of the 
tears by the inability to maintain these latter conditions. 

THE LIDS AND THEIR MOVEMENTS. 

The position and form of the lids and palpebral opening respond 
so clearly to nerve impulses and to such varied impulses that they afford 
diagnostic evidence of great value. But they are subject to marked 
normal and pathologic variations, due to alterations in the tissues of 




Fig. 9.— Thickening of the lids produced by trachoma. (Case of Dr. Jackson.) 



the lids themselves or of the structures that they cover, and these 
variations must be thoroughly understood before the symptomatic value 
of the appearances presented can be estimated. 

The lids form a freely movable curtain stretched across the orbital 
opening, loosely attached in all directions except to the nasal side in 
the region of the internal palpebral ligament. They are usually com- 
paratively thin and flaccid except for the normal elasticity of the skin 
and other tissues composing them. They lie upon the deeper tissues 
from which they largely take their outline. But the lids may become 



346 



THE EYE AND NERVOUS SYSTEM 



thickened so that the outline of the deeper structures is quite masked. 
Normally comparatively free from fat in the adult, they may become 
the seat of extensive deposits of adipose tissue, and usually do contain 
a good deal of it in early childhood. Their loose cellular tissue favors 
their rapid and excessive infiltration with serum, and they may become 
greatly thickened with more substantial and permanent exudate. 

Still more confusing are the changes produced by inflammation. 
These are quite common as a result of trachoma. In the chronic cases 
the deposits of connective tissue, besides thickening, bind the lid rigidly 
to adjoining tissues, greatly interfering with all of its movements. 
The effect produced is illustrated in Fig. 9. 

In this patient the alterations produced by chronic trachoma were 
chiefly confined to the right eye, so that the left serves as a standard 
of comparison. Syphilitic tarsitis causes a somewhat, but not exactly, 
similar alteration of appearance and function. It is generally bilateral. 

In the curtain of the lids the features especially influenced by 
nerve impulses are the palpebral opening and the superior and inferior 
orbitopalpebral folds. Of these folds the lower, between the lower lid 







Fig. 10.— Palpebral folds, a, brow ; £>, oculo-orbital fold, superior ; c, d, /, inferior folds ; e, cheek. 

(Wilbrand and Saenger.) 



and margin of the orbit, is most marked when the eye is turned strongly 
down. But even with that movement it may be quite indistinguishable, 
especially in children with much fat in the lids. On looking up there 
remain only the lines or creases, usually two, which mark the site of 
the fold. Its formation evidently depends upon traction of the inferior 
rectus, through its connections with the lid (Fig. 11). These folds are 
shown in Fig. 10. 

The upper fold h is well marked, although it varies greatly in 
appearance and becomes much deeper when the eye is rolled upward. 



THE LIDS AND THEIK MOVEMENTS 



347 



When the orbit is well filled with fat, it appears merely as a close fold 
in the skin. But when the adipose tissue is removed by age or wasting 
disease it becomes a broad fossa, which is the chief factor in producing 
the appearance of hollow or sunken eyes, in wasting disease. Below 
there are generally several smaller creases, c, d, f, divided into a tem- 
poral and a nasal group, which become emphasized as the eye is turned 
down and from or toward the nose. 

For a clear understanding of alterations brought about in the lids 
and palpebral opening, one must bear in mind the anatomic and physio- 



6b 6c 




Fig. 11.— Section of orbit. 1, skin of lid ; 2, orbicularis muscle ; 3, tarso-orbital fascia ; 4, con- 
junctival cul de sac; 5, tarsus; 6, elevator of upper lid; 6a, unstraited muscle connecting elevator 
with skin of lid ; 6b and 6c, connections of elevator with tarsus and conjunctiva ; 7, skin of lower 
lid ; 8, inferior rectus ; 8a, connection of lower lid. (Schwalbe). 



logic relations of the various structures of the orbit. To the margin 
of the orbit is attached a sort of connective tissue hammock, including 
part of the capsule of Tenon, denser connective tissue bands, and looser 
meshes. In this hammock lies the eyeball, drawn firmly back by the 
elastic tension of the recti muscles, and rotated or swung by their 
individual or combined contractions. The globe of the eye is not a 
ball turning in a fixed socket. The well-known fact that the centre 



348 THE EYE AND NEKVOUS SYSTEM 

of rotation is back of the centre of the eyeball means that the eyeball 
as a whole moves somewhat in the direction toward which it is rotated. 
On looking up it rises; on looking down it is depressed; on looking 
to the right or left it moves perceptibly in these directions. All such 
movements of the eyeball are closely followed by movements of the lids. 
The parts concerned are illustrated in Eig. 11. 

The harmony of movement of eyeball and lids is aided by the numer- 
ous bands that pass from the tendons of the recti and inferior oblique 
muscles to the lids ; so that the lid movements are partly due to the 
action of the muscles which turn the eyeball. 

A good example of how such harmony of movement is brought about 
is revealed by a careful study of the connections of the elevator of 
the upper lid and the superior rectus muscles. Besides a close anatomic 
association of the nuclei governing these two muscles and their habitual 
physiologic association of simultaneous use, slips from the tendon of 
insertion of each pass to the tendon of insertion of the other. 

Harmony of movement even of different parts of the upper lid is 
secured by such an arrangement of muscular insertions. The part of 
the skin covering the cartilage of the upper lid is normally kept rather 
closely applied to it, although the skin and cartilage are not firmly 
connected except at the lid margin. This close apposition of the skin 
to the cartilage is preserved, whatever the position of the lid and how- 
ever great the general redundancy of the skin. This has been ascribed 
to the elasticity of the tissue connecting the skin with the tarsus. But 
it is known that certain anterior fibres of Mueller's muscle in the upper 
lid pass through the orbicularis to be inserted in the skin of the lid. 
Dwight 1 points out that their function is to draw the skin to the fold 
above the tarsus when the lids are opened. Failure of this function 
causes atonic ptosis. 

The Palpebral Opening. — The palpebral opening varies in size 
and shape from a mere slit separating the closed lids to an almost 
circular aperture, the diameter of which approaches that of the eye- 
ball. The active factors in producing these changes are the constriction 
of the orbicularis tending to diminish the opening, and the contractions 
of the elevator of the upper lid and the superior and inferior recti 
muscles, with the attached unstriated muscles, which tend to retract 
the lids and so enlarge the opening. Passively, narrowing of the open- 
ing may be due to inflammatory or other deposits which tend to oppose 
the retractors of the lids or to weakness or paralysis of these retractors. 

1 System of Ophthalmology, edited by Norris and Oliver, vol. i. p. 92. 



THE LIDS AND THEIK MOVEMENTS 349 

Widening of the palpebral aperture is passively favored by paralysis 
of the orbicularis, or by enlargement of the eyeball, or its being pushed 
forward by pressure from behind, or by cicatricial adhesions preventing 
the normal closure of the lids. 

The nerves directly concerned in the changes of form of the opening 
are the facial, distributed to the orbicularis ; the oculomotor, supplying 
the recti and the elevator of the upper lid ; and the sympathetic, which 
is supposed to innervate the unstriated fibres of Mueller's muscles of 
the lids. The palpebral opening is generally more nearly circular and 
relatively wider in childhood. Excitement and probably the influence 
of general cold tend to widen the aperture. Stimulation or irritation 
of the cervical sympathetic and the local application of cocaine have 
the same influence. On the other hand, age, weariness, stupor, paraly- 
sis of the sympathetic or its division or excision, tend to diminish the 
palpebral opening. 

Exophthalmos widens and enophthalmos causes narrowing of the 
aperture. But retraction of the lids and widening of the palpebral 
opening causes an appearance of exophthalmos, while narrowing of 
the opening causes an appearance of enophthalmos. Unfortunately, 
the prominence of the eye-ball in the orbit and the actual variations 
in its position with reference to the plane of the orbital opening have 
rarely been subjected to actual measurement ; so that there may often 
have been retraction of the lids causing an appearance of exophthalmos, 
when it was supposed that widening of the palpebral opening had been 
caused by exophthalmos. Certainly, eyes that appear to be protruded 
or retracted often prove not to have changed their relations with the 
orbital margin. 

On the other hand, actual protrusion of the eyeball may be accom- 
panied by swelling or relaxation of the lids leading to narrowing of 
the licl opening, which partly masks the exophthalmos. In cases of 
exophthalmos slowly developed by the increase of an orbital tumor 
the lids may be enormously enlarged. 

Winking. — Normal winking is a rhythmical closure of the palpe- 
bral opening by the contraction of the tarsal portion of the orbicularis, 
as a reflex probably to a need for renewed moisture of the corneal sur- 
face, a sort of thirst of the part, which usually does not affect conscious- 
ness. The afferent impulse passes through the fifth, the efferent through 
the seventh nerve. Winking is repeated from two to five times a min- 
ute. Howe, 1 who studied it by photography, finds that the closing 



1 Transactions of the American Ophthal. Society, 1904. 



350 



THE EYE AND NEKVOUS SYSTEM 



of the lids occupies one-twentieth to one-tenth of a second. They 
remain closed two- or three-tenths of a second, and the lifting of the 
lid again takes one- or two-tenths of a second. Slow lifting, he thinks, 
may be a valuable sign of paresis of the elevator of the lid. 

Lid Signs. — Although the normal variations in the palpebral open- 
ing make it difficult to judge at first sight whether or not the lids are 
abnormally separated, any change of the kind is likely to be noticed, 
in the expression of the eyes, by those familiar with the patient. When 
the lids are separated too widely it gives the eye a staring expression. 
When this is habitually present the retraction is probably due to spasm 
of the unstriated muscle-fibres of the lids innervated through the sym- 




Fig. 12.— Graefe's sign : exposure of sclera on looking down. From a case of exophthalmic goitre. 

(Wilbrand and Saenger.) 

pathetic. This has been called Dalrymple's sign, It may cause ex- 
posure of the sclera both above and below the cornea. It can be imi- 
tated by the application to the eye of cocaine, which produces a similar 
spasm of Mueller's muscle that lasts considerably longer than the anaes- 
thesia, but hardly so long as the dilatation of the pupil produced by 
this drug. (Compare Page 835.) 

The normal movements of the lids with the eyeball have already 
been referred to. The movements of the upper lid, upward or down- 
ward as the eye is turned up or down, is by far the most extensive of 
these lid movements, and in health is very accurately co-ordinated with 
the movements of the globe. In disease the downward movement of 
the lid, when the eye is turned down, may become very defective or 
altogether wanting. The upper lid remaining elevated and retracted 



THE LIDS AND THEIE MOVEMENTS 351 

when the eye is turned downward causes an appearance that is strikingly 
abnormal. This is shown in Fig. 12. 

This is named for its discoverer, the Graefe sign. It is easily 
confused with the Dalrymple sign. When the eyes are turned down 
they are really too widely open. But if the Graefe sign alone he present 
it can be seen that there is no special retraction of the lower lid ; and 
when the eyes are turned up there is no abnormal widening of the 
palpebral opening. In practice the two signs are very likely to be 
seen together, there being both a widening of the opening — Dalrymple's 
sign — and a failure of the upper lid to move down with the eyeball — 
Graefe's sign. The disturbance of the co-ordination of the lid move- 
ment and eye movement is probably a failure of function in the nuclei 
controlling these movements ; or the effect of some abnormal impulse 
or lack of control from the higher centres. (Compare Page 833.) 

An impairment of a closely related function, a disturbance of the 
nervous mechanism concerned in the reflex movements of the lids, leads 
to a third lid sign named for Stellwag. Stellwag' s sign consists of 
infrequent, irregular, and incomplete winking, occurring independently 
of any paresis of the seventh nerve or defective sensibility in the cornea 
or conjunctiva. The eye exhibiting it may remain a minute or more 
without winking. Then there may be repeated inperfect winking move- 
ments. Sometimes the movements will be repeated at nearly the usual 
intervals (two to five times a minute). But most of the closures of 
the eye will be incomplete. Where Stellwag' s sign is well marked the 
lid movement of winking will be noticeably hesitating and irregular 
in character. The sign of Stellwag is commonly associated with those 
of Graefe and Dalrymple. (Compare Page 834.) 

Lid Reflexes. — The reflex closure of the lids to a touch of a foreign 
body upon the cornea or conjunctiva felt through the fifth nerve, or 
to the impression of something approaching the eye transmitted through 
the retina and optic nerve, is very prompt, certain, and quite beyond 
the control of the will. Any one who frequently has to instil fluids 
into the eye or make applications to the conjunctiva will appreciate 
the constancy and force of this reflex. Touching or striking the skin 
of the lids or neighboring parts of the face will produce a movement 
toward closure of the palpebral opening. 

A special sensorimotor reflex of the lids has been described by 
McCarthy, who calls it the supraorbital reflex. To elicit this reflex 
the patient should relax the muscles about the eyes. Then a slight 
tap of the percussion hammer is given high on the forehead. This 
usually excites a general movement of closure of the lids. But the 



352 THE EYE AND NEKVOUS SYSTEM 

action to which McCarthy calls attention is a quick fibrillary contrac- 
tion of the lower half of the orbicularis palpebrarum. This reflex is 
absent after division, or disease destroying the function of the orbital 
nerve, and in all cases of peripheral paralysis of the facial nerve. (See 
Page 916.) 

Widening of the Palpebral Opening by Contracture of the Ele- 
vator. — Besides the opening of the eye produced by the healthy exercise 
of the function of the elevator of the upper lid and that due to retraction 
of the lids by Mueller's muscle, which is discussed elsewhere, abnormal 
widening of the palpebral fissure may occur as a reflex or choreiform 
symptom. Posey 1 has reported a case of rhythmical widening about 
fifteen to twenty times a minute, which ceased after the use of atropine 
for the measurement of ametropia. Other cases of similar peculiar 
tremor of the lids have been reported in connection with exophthalmic 
goitre, ophthalmoplegia externa, and facial paralysis. Secondary con- 
tracture of the elevator of the upper lid sometimes occurs after facial 
paralysis of either peripheral or central origin. It leads to abnormal 
widening of the opening, closely resembling the Dalrymple sign. 

Lagophthalmos. — Continuous exposure of the eyeball by inability 
to close the lids may be due to cicatricial contraction deforming the lids 
or drawing them away from their normal position and binding them 
down so as to interfere with their function. In such cases the scars 
responsible for the deformity are immediately evident upon inspection. 

In elderly people with great relaxation of tissue and general mus- 
cular weakness, there may be inability to close the eyes fully. In cases 
of this kind there is often some conjunctival inflammation and swell- 
ing, so that ectropion of the lower lid occurs and may give more trouble 
than the exposure of the eyeball. 

Complete facial paralysis of peripheral origin is always attended 
with inability to close the eye by voluntary contraction of the orbicu- 
laris, although the lids can easily be drawn together with the fingers. 
With attempts at forcible closure of the lids, however, the eyeball is 
normally rolled up so that the cornea is greatly protected from injury; 
and in many cases the inability to close the lids by winking is replaced 
by frequent movements of the eyeball upward, which secures the 
moistening of the cornea and removal of foreign bodies from its sur- 
face. If, however, these movements are not made with sufficient fre- 
quency, or, if in a case of long standing, secondary contracture of the 
upper lid increases the exposure of the eye-ball, there is great danger 

1 Journal of Nervous and Mental Disease, July, 1902. 



THE LIDS AND THEIE MOVEMENTS 353 

of serious disease of the cornea. This is particularly likely to occur 
in elderly or poorly nourished patients or when the sensibility of the 
cornea is impaired. 

Exposure Keratitis. — Lagophthalmic keratitis arises from the un- 
due drying of the exposed portion of the cornea. It begins with dis- 
turbance of the epithelium and superficial ulceration near the lower 
corneal margin. Infection occurring through the channels thus opened 
to it leads to purulent infiltration of the subjacent tissue. The infil- 
tration runs a comparatively chronic course and is marked by slight 
inflammatory reaction, as compared with that which usually attends 
an infective keratitis of equal extent and gravity. If unchecked, the 
process goes on to perforation of the cornea, extensive adherent leu- 
coma, and practical loss of usefulness of the eye. 

The treatment of this condition must include careful protection of 
the eye from irritants, as by dressings that will close the lids and hold 
them shut. If corneal infection requiring active treatment has not 
occurred or has been overcome, the most efficient means of protecting 
the eye is by uniting the margins of the lids. These may be freshened 
and brought together by sutures throughout a part of the palpebral 
fissure. Usually the tarsorraphy is done at the outer canthus, but in 
some cases a better effect will be obtained by uniting the lid margins 
near the region of the lachrymal puncta. In this way we secure the 
proper drainage of tears and leave the temporal portion of the palpebral 
opening free, preserving part of the field of vision. The exact extent 
and location of the union of the lids must be determined by study of 
the individual case. If union of the lids be resorted to early, before 
infection has occurred, the prognosis is quite favorable. And if at a 
subsequent time recovery of the power of the orbicularis occurs, the 
connection between the lids can readily be divided. 

Ptosis. — The conditions apart from abnormal innervation which 
cause narrowing of the palpebral opening have already been referred 
to (page 347). Congenital ptosis, hysterical ptosis, and ptosis due 
to disease of the oculomotor nerve, or of the central nervous system, are 
discussed in Chapter V. It remains here to consider the general 
characteristics of ptosis, a few forms arising from local conditions, 
and a brief outline of the treatment. In a general way ptosis is due 
to relaxation of the elevator of the lid or to a more or less permanent 
contracture of the orbicularis. 

Relaxation of the elevator is attended with comparative smooth- 
ness of the skin of the lid and partial obliteration of the upper oculo- 
orbital fold (Fig. 13). Complete paralysis of the elevator is shown 
23 



354 THE EYE AiSTD NEKVOUS SYSTEM 

by the fact that on looking upward there is no deeping of this 
fold. Contraction of the orbicularis tends to cause wrinkling of the 
skin of the lids and adjoining parts. 

Temporary Ptosis. — Any inflammatory disease of the eye which con- 
tinues many days is liable to cause narrowing of the palpebral opening. 
This occurs quite independently of any exudation into the lids that 
might mechanically oppose their full opening. It is at first an effort 
to protect the inflamed eye from light or irritation. It is most notice- 
able in connection with chronic inflammation affecting but one eye. 
Such a ptosis may be continued indefinitely to diminish the light fall- 
ing on a nebula left by corneal inflammation. But even when no such 




Fig. 13.— Ptosis: obliteration of palpebro-orbital fold. (Case of Dr. Jackson.) 

sequel remains and the inflammation has entirely passed away, nerve 
habit may continue for a time a drooping of the lid. This drooping, 
however, will slowly diminish, and in the course of weeks or months 
will entirely disappear. The process of recovery may be hastened by 
massage and voluntary efforts to open the eye widely. Possibly electric 
stimulation of the muscle may be of benefit. But such ptosis may be 
due to contracture of the orbicularis rather than to relaxation of the 
elevator, and injudicious use of electricity in such a case would be 
pretty certain to do more harm than good. 

Another form of temporary ptosis is seen in persons with organic 
disease of the central nervous system. Sometimes the upper lid will 
droop for a few hours, sometimes for days or weeks, although the patient 
can usually open one eye widely by special effort and there may be 
no other evidence of oculomotor paresis. This form depends on relax- 
ation of the elevator. The prognosis regarding it is very uncertain, 



THE LIDS AND THEIR MOVEMENTS 355 

but it may disappear permanently, even after it has been troublesome 
for a long time. 

. Not a few persons complain of special difficulty in opening the 
eyes after particularly sound sleep. Although there is no adhesion 
of the lids and the patient seems to have fully regained consciousness, 
there remains a sense of inability to move the lids. This may be com- 
pared with the feeling experienced in a nightmare, of inability to per- 
form some act in spite of the strongest desire to do it. The specially 
intimate connection of closure of the eyes with the phenomena of sleep 
is probably the basis of this kind of ptosis. 

Atonic Ptosis. — Without loss of power in the elevator of the upper 
lid, and with normal movement of the tarsal cartilage, the skin of the 
lid may form a fold hanging down over the lid margin and roots of 
the lashes, so as to limit materially the field of vision. This condition 




Fig. 14.— Swelling of lids following traumatism. (Case of Dr. Jackson.) 

may be simulated by a redundancy of tissue. In the older works it 
was called ptosis from hypertrophy, while atonic ptosis (Mackenzie) 
was used to designate what would now be known as hysterical ptosis. 
Anatomical examination of the most typical cases of this class has 
shown atrophy rather than hypertrophy of the . tissue. Hotz pointed 
out that the condition was due to impairment or destruction of the 
normal relation between the skin of the upper lid and the tarsus. So 
that when the tarsus was retracted the relaxed skin formed a fold which 
fell over its margin. The connection which Dwight has pointed out, 
between a part of the skin and a slip of Mueller's muscle connecting 
it with the tendon of the elevator, suggests that this form of ptosis 
may be due to relaxation of this muscle, and properly rank as a form 



356 THE EYE AKD NERVOUS SYSTEM 

of ptosis from disease of the sympathetic. Its treatment will be men- 
tioned under the operative treatment of ptosis. 

Traumatic Ptosis.. — Scars involving the lids may cause ptosis, but 
this form need not be described here. Attention is called to it that 
inability to raise the lids from this cause shall not be confused with 
that due to impaired nerve action. Penetrating wounds of the orbit 
may involve the branch of the oculomotor nerve which supplies the 
elevator muscle. But they are more likely to do direct injury to the 
muscle. If the wound be extensive the involvement of the muscle in 
the resulting scar is likely to produce permanent disability. Ptosis 
from this cause is illustrated in Eig. 14. Some power of movement 
may be regained with the subsidence of the inflammatory swelling, but 
some permanent impairment is the rule after such injuries. 

Operative Treatment of Ptosis. — Without discussing the numerous 
ptosis operations in detail, they may be mentioned with the principle 
involved in each. For traumatic ptosis a plastic operation, planned 
to meet the indications of the particular injury, may be required. The 
object aimed at, in such an operation, will be the freeing of the lid from 
abnormal restriction of its upward movement. 

Eor ptosis due to hypertrophy either of the skin of the lid or sub- 
cutaneous tissue, the removal of the proper quantity of hypertrophied 
tissue is the rational procedure. But for ptosis not due to actual hyper- 
trophy, even for that which is evidently associated with great relaxa- 
tion, the mere removal of tissue is likely in the end to prove unsatis- 
factory. Excision of an elliptical piece of skin may be of some benefit 
in the slighter degrees of paretic ptosis. But contraction thus brought 
about tends to obliterate the fold between the lid and upper margin 
of the orbit, causing a deformity as noticeable as that which it removes. 
Excision of an excessively large flap, in addition to causing this new 
deformity, may produce a dangerous lagophthalmos, while it still fails 
to remove the ptosis on turning the eye upward. 

A more reliable operation and one that does not cause the same 
obliteration of the oculomotor fold is that of Wilder. He exposes the 
tarso-orbital fascia by making an incision just below the brow and dis- 
secting a flap down to the tarsus. Sutures introduced through the mar- 
gin of the tarsus are carried in and out through the fascia, as gathering 
stitches, up to the line of incision, drawn upon sufficiently to give the 
proper elevation of the lid, tied and cut short. The incision is closed by 
skin sutures. This gathers or folds the fascia upon itself, and leaves an 
almost imperceptible scar. 

Atonic ptosis, which has often been taken for hypertrophic and 



THE LIDS AKD THEIE MOVEMEXTS 357 

subjected to excision, should be treated as suggested by Hotz. An 
incision in the general direction of the lid margin, two or three milli- 
metres from the margins at its ends, and somewhat farther from the 
margin in the centre, is made through the skin and down to the cartilage. 
This incision is closed by sutures which pass first through the lower lip 
of the incision and then through the upper margin of the tarsus. These 
sutures when tightened stretch the lower flap of skin and unite it firmly 
to the tarsus, so that the subsequent folding or falling below the lid 
margin would be impossible. 

For ptosis due to paresis of the elevator, operations for the advance- 
ment, resection, or folding (tucking) of the tendon of the elevator are 
appropriate. In Eversbusch's folding operation the tendon is exposed 
four millimetres above its insertion into the tarsus. A suture with a 
needle on each end is applied to the tendon, and the needles carried 




Fig. 15.— Complete bilateral ptosis. Maximum elevation of the lids by contraction 
of the frontalis. (Case of Dr. Jackson.) 

between the skin and tarsus to the lid margin, where they are brought 
out and the ends of the suture tied over a bit of rubber tubing. Three 
of these sutures are placed : one at the centre and one at each end of 
the lid. The tightening of these sutures folds the tendon down over 
the tarsus. 

Snellen's operation is a resection. The tendon is exposed and three 
or four needles thrust through it the necessary distance back 'from the 
tarsal margin. The end of the tendon is then excised by parallel in- 
cisions along the tarsal margin and in front of the needles. The nee- 
dles being brought through the upper margin of the tarsus, and the 
threads tightened, the desired shortening of the tendon is secured. 

"Wolff does resection by exposing the tendon and raising it on two 
strabismus hooks. The sutures are introduced through the tendon just 



358 THE EYE AND NERVOUS SYSTEM 

below the upper hook. The end of the tendon is then cut away from 
the tarsus, and the sutures passed through the insertion of the tendon 
into the tarsus. 

De Lapersonne practises advancement of the tendon of the levator, 
with or without excision, by suturing the tendon upon the outer surface 
of the tarsus instead of to its upper margin. 

For paralysis of the elevator which is complete and permanent, the 
only operations worthy of much attention are those which give power 
to raise the lid by attaching it to some other muscle which may thus 
be made to perform a vicarious function. The effort of nature in these 
cases is to drag the lid upward through its skin connection with the 
frontalis. The result achieved is shown in Eig. 15. 

To give a more firm and efficient connection between the lid and 
frontalis, Dransart sought to produce a scar by introducing cat-gut 
sutures subcutaneously from the lid to the brow. To increase the 
cicatricial change, Pagenstecher substituted silk for the catgut. This 
was tied over a bit of rubber tubing above the brow, allowing it to be 
shortened from time to time as seemed desirable. Mules, finding the 
scars secured by such measures left much to be desired in the way of 
permanence and rigidity, introduced a silver wire by which the desired 
elevation of the lid was secured. The wire was then knotted and the 
knot buried above the brow. Worth has used kangaroo tendon for this 
purpose. Harman, instead of silver wire, has employed the light, 
woven gold chain sometimes used for an eyeglass-cord. This being 
thoroughly sterilized and buried becomes intimately incorporated with 
scar-tissue, and, being entirely flexible, is less likely to cause subsequent 
disturbance than is the silver wire. 

The connection of the lid and frontalis tendon by transplanted tissue 
was effected by Panas in the operation which bears his name. In this 
operation an incision is made parallel to the brow above it and another 
below. The tissue between them is undermined. A broad, tongue- 
shaped flap of skin and subcutaneous tissue is dissected up from the 
lid and oculomotor fold, carried up under the bridge of tissue, and made 
fast at the insertion of the frontalis muscle into the skin of the brow. 

Panas's operation has been the subject of slight modifications by 
individual operators; but is essentially the operation most commonly 
done to-day for this form of ptosis. All operations that aim to connect 
the lid with the frontalis tendon, whether by thread, wire, scar-tissue, 
or flap, have these defects : the oculo-orbital fold is more or less obliter- 
ated and the movement of the lid is at best accomplished by an abnor- 
mal contraction of the frontalis. 



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THE LIDS AND THEIR MOVEMENTS 359 

The muscle which normally contracts in closest association with the 
elevator of the upper lid is the superior rectus. In a normal state every 
contraction or relaxation of the one muscle is accompanied by a similar 
contraction or relaxation of the other; except that when the eye is 
strongly closed the elevator of the lid is relaxed, but the eyeball is rolled 
up by contraction of the rectus. 

Motais devised the operation of connecting a portion of the tendon 
of insertion of the rectus with the tarsus. An incision being made 
through the conjunctiva and subconjunctival tissue down to the tendon 
of the superior rectus on the eyeball, and in the direction of the tendon, 
is carried back to the retrotarsal fold and through the palpebral con- 
junctiva to the tendon of the elevator of the lid. The rectus tendon is 
isolated, split into three parts, and the middle part separated from 
the insertion into the sclera and by two sutures carried up and inserted 
in the upper margin and external surface of the tarsus. 

Thus treated, the superior rectus muscle quickly gains power to 
move both the lid and eyeball. So that henceforth the lid moves nor- 
mally with the globe of the eye, except that on forcible closure of the 
lids the upper lid is pulled up with the effort of the superior rectus to 
roll the eye upward. Motais' s operation causes no visible scar and 
tends to restore to normal the oculo-orbital fold. It gives to the lid 
its most important and useful movements. But it does not confer 
the power of retraction of the lid independently of movements of the 
eyeball. 



CHAPTEE XVIII. 
DISEASES OF THE EETINA AND OPTIC NEEVE. 

By H. V. WIEDEMANN, M. D. 

Ophthalmoscopy. 

Since the possibility of examining the interior of the eye by means 

of the ophthalmoscope was demonstrated by von Helmholtz in 1851, 

much light has been thrown, by the use of this instrument, upon various 



Fig. 1. — The Loring ophthalmoscope. 

general as well as ocular diseases, and the diagnosis of many general 
disorders and diseases of the nervous system has been greatly simplified. 
Though the instrument, as originally devised by von Helmholtz, pos- 
sessed all the properties essential to a thorough examination of the 
fundus, its elaboration and improvement by Reute, Liebreich, Loring, 

and Morton, have added greatly to the facility with which it may be 
360 



OPHTHALMOSCOPY 361 

used and have rendered it possible for the expert to estimate very accu- 
rately the refraction of the eye. 

The accompanying cuts illustrate the Loring ophthalmoscope (Fig. 
1), the most popular instrument in this country, and a model of an elec- 
tric-lighted ophthalmoscope (Fig. 2), which is especially serviceable for 
bedside work. Pig. 3 illustrates a model of a stationary ophthalmoscope. 
This instrument is a valuable adjunct to the hand ophthalmoscope, as it 
gives an extended view of the magnified and inverted image, is most 
useful for demonstration to students, and for the purpose of reproduc- 
ing fundus pictures by drawing or photography. 

In ophthalmoscopy the illumination should be steady and uniform, 
and while the fundus may be seen on bright days with ordinary day- 
light passing through a small aperture into a darkened room, this source 
of illumination can be used only occasionally, and thus most of our con- 
ceptions of the fundus are based upon observation by yellow artificial 
light; a candle or lamp flame, a gas jet or an electric light covered by 
frosted glass, being utilized for the purpose. In the performance of 
all ordinary tests with the ophthalmoscope, both the observer and the 
patient should be comfortably seated. If the patient be bedridden 
this is impossible and the examination must often be conducted from 
a constrained posture. Although this is often fatiguing, the surgeon 
should not desist until he has satisfied himself that he has accomplished 
all that was possible under the circumstances. 

The interior of the eye may be viewed with the ophthalmoscope by 
either the direct or indirect method. The former is to be preferred, for 
although somewhat more difficult of performance, it permits of a study 
of the fundus under a much greater magnification, the back part of 
the eye being p magnified 14 diameters by the direct method, and but 
4 by the indirect, and at the same time it enables the observer to obtain 
a very accurate idea of the refraction of the eye. The indirect method 
is only to be preferred in high myopia and in cases where the media of 
the eye are hazy. Before resorting to the study of the interior of 
the eye, the observer should always obtain as much information as is 
possible of the media by means of transmitted light. For this purpose, 
the observer, at a distance of about 30 centimetres, flashes the light 
which is placed behind and slightly to the side of the patient's head 
into the eye, by means of the mirror of the ophthalmoscope. A faint 
pinkish-red glow will be seen to replace the blackness of the pupil. This 
is known as the fundus reflex, and if the glare be unbroken and uni- 
formly bright, even when the eye is moved about through the different 
meridians, the media are clear and no opacities obscure vision. 



362 THE EYE AND NEKVOUS SYSTEM 

The Direct Method. — Having satisfied himself of the condition 
of the media, the observer now brings his chair alongside the patient's, 
and concentrating the light upon the cornea, by means of the ophthal- 
moscope which he holds in close apposition with his own eye, he en- 
deavors to approach as closely as possible to the patient's eye. If he 
will now relax his accommodation, by fancying that what he is now 
regarding is situated far in the distance, and request the patient to 
look straight forward into the darkness, for ophthalmoscopy should 
preferably be performed in a darkened room, the observer will now 
see the vessels of the retina and the head of the optic nerve. 

Attention should be paid next to the macular region, which may 
be brought into view by slight movements of the patient's eye to the 
right or left, and finally the patient should look up, down, in and out, 
as requested by the observer, bringing the various parts of the fundus 
into view. Observations are made of the color of the fundus and of 
the optic disk; of changes in the elevation of the disk by focussing it 
and the surrounding areas; of changes in the blood-vessels, hemor- 
rhages, pigment deposits, and degenerative areas. 

The Indirect Method. — In the application of this method, the ob- 
server stations himself in a position corresponding to that assumed 
in the test by transmitted light and throws the light into the eye 
under examination by the concave mirror of the ophthalmoscope. A 
convex lens of +14.00 D. strength is then held before the eye to be 
examined, and an aerial image obtained of that portion of the fundus 
illuminated by the reflected light. In case the details of the fundus 
are not plainly seen at first, the object lens should be slightly moved 
to or from the eye, as a compensation for the patient's error of refrac- 
tion. The image obtained by the indirect method is inverted, and it 
must be remembered that the upper part of the image corresponds to 
the lower part of the eye-ground and the right side of the image to the 
left of the eye-ground. 

The Normal Fundus. — The normal eye presents many variations, 
and an extended experience is frequently necessary to distinguish con- 
genital variations from changes wrought by disease. 

The Fundus Reflex. — The color in normal eyes is bright pink or 
red, due to the reflection from the pigment of the retina, choroid, and 
the choroidal vessels. In the albino there is no fundus pigment, and 
the reflex is that of the choroidal and retinal vessels with the sclera 
as the background, and is a light pink. In the negro the reflex is gray, 
on account of absorption of the light rays by the abundant pigment. 
(See Plate III.) 








Fig. 6. — Region of optic disk showing opaque nerve-fibres and two cilioretinal 
vessels (from photograph). 



OPHTHALMOSCOPY 



363 



The Papilla. — The optic disk or nerve-head is about 1.75 millimetres 
in diameter, appearing under the ophthalmoscope enlarged about fifteen 
times, and hence from 10 to 15 millimetres (one-half to six-eighths 
inches) in width. It is round or oval, with clear-cut margins ; its 
centre is whitish, though the connective tissue lace-work supporting 
the nerve-fibres and the lamina cribosa are a mottled gray. The 
periphery of the disk is pink, owing to fine blood-vessels in its structure 
and on its surface. (Plate III.) There is usually an excavation in 
the nerve-head, due to the entrance of the retinal vessels ; in some cases 
this is quite small, while in others it is so large that it embraces nearly 
the entire papilla. This excavation is physiological and should not 
be confused with that of glaucoma, which occupies the whole of the 
disk, or that which is consecutive to optic nerve atrophy. 

The Blood-Vessels. — The retinal vessels leave the disk slightly to 
:the nasal side of the centre and divide into two or more main stems, — 




Fig. 7. —Diagram of the blood-vessels of the human retina (Leber, after Jaeger) , — ans, vns, superior 
nasal artery and vein; ats, vts, superior temporal artery and vein ; ani, vni, inferior nasal vessels; oft", 
vti, inferior temporal vessels ; ame, vme, median vessels ; am, vm, macular vessels. 

the superior and inferior retinal, sending numerous fine branches to 
the adjacent retina. Just above the disk each trunk bifurcates into 
branches which pursue a semicircular course, curving round towards 
the temporal and nasal parts of the eye. The veins follow the arteries 
in their course more or less closely, but may be differentiated from 
them by being darker in color, and being about one-third larger. Both 



364 THE EYE AND NEKVOUS SYSTEM 

arteries and veins have a light streak along the centre, which is fainter 
upon the veins, the reflex streak. A distinct pulse occurs physiologi- 
cally in the veins upon the head of the nerve, but pulsation in the 
arteries is always pathological. The clinician should acquire the 
habit of observing the retinal blood-vessels with great care, for their 
condition is frequently indicative of the state of the blood-vessels else- 
where throughout the system; and on account of the possibility of 
viewing the actual blood-column itself an exceptional opportunity is 
offered of diagnosing pathological conditions of the blood. 

The Macula. — The yellow spot, which is situated about 2 disk diam- 
eters (3 mm.) towards the temporal side of the nerve in the horizontal 
meridian of the fundus, is the most sensitive part of the retina. It 
consists of an apparently yellow spot, surrounded by a circular or oval 
area, the foveal region, which is darker in color than the rest of the 
fundus and about the size of the disk. The centre of the macula is 
known as the fovea centralis, and appears as a glistening reflex. The 
blood-vessels do not cross the fovea but converge towards it. In order 
to obtain the best view of the macula, the ophthalmoscope should be 
slowly moved upward and inward, while the observer brings his line 
of vision to a point about two to two and a half disk diameters out- 
ward from the disk. When the pupil is dilated the macula may be seen 
by having the patient gaze directly into the sight-hole of the mirror. 
The periphery of the eye-ground should be carefully studied ; and in 
order that no part of it may escape, it is well for the student to follow 
each branch of the central artery of the retina as far forwards as 
possible. 

Ophthalmoscopic Signs of General and Nervous Diseases. 

Certain conditions of the fundus oculi are of preeminent interest 
and value in the diagnosis of various general and nervous diseases : 

(1) Sensory affections : hyperesthesia, anaesthesia. 

(2) Vascular lesions : 

(a) Changes in the blood-vessels: anaemia, hyperaemia, 

pulsation, oedema. 
(&) Inflammation of the vessel-walls. 

(c) Hemorrhages from the vessels. 

(d) Stoppage of the vessels: embolism, thrombosis. 

(3) Inflammation of the retina. 

(4) Inflammation and oedema of the optic nerve: optic neuritis, 

choked disk. 

(5) Atrophy of the optic nerve. 



PLATE III. 




c D 

Varieties of the Normal Fundus.— A. The albinotic fundus; albino and light blonde (after 
Greeff, modified by H. V. W.) ; B. The tessellated fundus ; brunette (after Greeff, modified by H. V. W.) ; 
C. The negroid fundus; Negro (H. V. W.) ; D. The yellow fundus; Chinese (after Oeller, modified by 
H. V. W.) 



DISEASES OF THE KETItfA 



365 



These lesions are also described under headings descriptive of various 
diseases, — i.e., as syphilitic, albuminuric, diabetic, and anaemic retinitis. 

(i) SENSORY AFFECTIONS. 

Hyperesthesia is due to retinal irritation and hyperemia giving rise 
to photophobia, asthenopia, and blepharospasm. It is usually origi- 
nated by local affections, such as uncorrected ametropia, presbyopia, 
and muscular imbalance, but it may likewise occur in neurasthenia, 
gout and oxaluria. Anaesthesia of the retina is characterized by diminu- 
tion of the central visual acuity and contraction of the visual field, or 
misplacement of the boundaries of the field or by changes in the bound- 



Right 




Fig. 8.— Hysteric Field, simulating Tempo- Fig. 9.— Hysteric Field, showing Progres- 

ral hemia.nopia with great reduction of vis- sive contraction (fatigue type) and over- 
ual acuity. lapping of color field. 

1. Form limits ; 2. Red, taken immediately afterwards ; 3. Red, taken 5 minutes later ; 4, Form, taken 
5 minutes still later. 

aries for color vision (Figs. 8-9). It is a prominent symptom of 
hysteria and neurasthenia, is present in exhausting fevers and in con- 
valescence, and in women with pelvic diseases. It may be induced 
by traumatism and by suggestion. The ophthalmoscope usually reveals 
hyperemia or anaemia of the nerve-head and retina. 



(2) VASCULAR LESIONS. 

Anaemia of the disk, as well as the retina, expresses impaired nutri- 
tion. It is found in ischemia and atrophy due to functional disturb- 
ances and blood diseases, and after severe hemorrhages. With the oph- 
thalmoscope the arteries are seen to be apparently contracted, carry 
less blood, and are of a light color ; the veins are full and even tortuous. 



366 THE EYE AND NERVOUS SYSTEM 

The papilla is pale, and there are, as a rule, no small vessels on its sur- 
face. (Plate IV., A.) 

Hyperemia evidences itself most prominently in the papilla, and is 
characterized by increase in the number of visible blood-vessels and 
by dilatation and tortuosity of the veins. True pathological increase 
of the vessels is rare, except in the beginning of optic neuritis or accom- 
panying retinitis. The retinal vessels become abnormally vascular if 
the cause be mechanical obstruction to the blood, either going from or 
coming to the eye, the chorioid likewise shows congestion, and the pink 
color of the healthy fundus assumes a dark uniform red. Physiologic 
variations in the color of the nerve-head, on account of the contrast 
afforded by the varying amount of pigment in the retinal layer of the 
chorioid, should always be considered in making a diagnosis. 

Causes. — Anaemia and hyperemia are symptoms of both constitu- 
tional and local diseases. Among the constitutional causes may be 
mentioned retention in the blood of excretory products, degenerative 
diseases of the cerebral cortex, as epilepsy and general paralysis, hyper- 
trophied and overacting heart from obstruction of the circulation, or 
thrombosis, organic disease of the abdominal organs, uterine disease, 
the beginning and cessation of menstruation, and finally the circulation 
of malarial or other toxic materials in the blood. Locally, the usual 
causes are chronic inflammation of the uveal tract and errors of refrac- 
tion, especially hyperopia and astigmatism, with accommodative strain. 

Treatment. — The treatment consists in the removal of the cause, with 
the use of atropine and the correction of errors of refraction, conjoined 
with the administration of alteratives and the stimulation of the excre- 
tory organs. 

Abnormalities in the size of the retinal arteries and veins may 
occur as a result of some intra-ocular inflammation, or may portend 
some serious affection behind the eyeball. 

Atrophy of the vessels occurs in association with atrophy of the retina 
and nerve. Varicosity of the veins may be congenital. 

Pulsation of the retinal veins is frequently physiological, whereas 
pulsation of the arteries is always pathological, indicating an enlarged 
heart, some vascular affection of the head or orbit, or glaucoma. 

CEdema of the retina may be general or limited to a certain por- 
tion. It manifests itself as an opaque, slightly elevated area, with the 
blood-vessels running over and through it. The vessels are decidedly 
dark, but lose their central light streak, and distinction between 
veins and arteries is difficult. . In general oedema the retina is infil- 
trated in all its layers. (Plate IV., A and E, and V., A and B.) 



PLATE IV. 






E F 

Inflammations of the Retina. — A. (Edema in Pernicious Anaemia (Oliver) : B. Leukaemie Retinitis 

(Oliver); C. Albuminuric Retinitis and Neuritis occurring in Pregnancy (H. V. W.) : D. Albuminuric 
Retinitis in the Negro (H. V. W.) ; E. Syphilitic Retinitis (Haab) ; F. Atrophy of Retina, Nerve and 
Chorioid following Disseminated Chorioretinitis Luetica (Oeller). 



DISEASES OF THE RETINA 367 

Hemorrhages and exudation may appear in any part of the retina. 
(Edema is met with in syphilitic retinitis ; it may be a forerunner of 
retinal detachment, of tumor of the retina or chorioicl ; it may he occa- 
sioned by traumatism, or may arise from exposure to bright light or 
electric flashes. (Edema of the disk occurs in the first stage of neuritis. 

Inflammation of the Vessel Walls. — Vasculitis and Perivasculitis 

These conditions are, as a rule, due to arteriosclerosis or syphilis, or 
occur in degenerative diseases of the cerebral cortex, and are attended 
by other lesions in the optic nerve and retina as well as the general 
system. (Plate V.) (See Chapter XL) 

Perivasculitis is an inflammation of the external layers and fibrous 
tissues surrounding the blood-vessels. It is most commonly met with 
in retinitis, but may exist separately. The blood-vessels are swollen in 
places, but are eventually replaced by fibrous cords. (Plate V., E.) 

Angeoid streaks are cicatricial changes in the retina, usually the 
remains of hemorrhages, though they may mark a previous detachment 
of the retina. They course from the disk to the periphery, are whitish 
in the centre, and are bordered by black lines. 

Hemorrhages from the Vessels, — Apoplexy of the retina occurs most 
commonly in elderly people with degenerated arteries. (Plate V., E.) 
It is met with in cardiac hypertrophy from valvular disease, ulcerative 
endocarditis, chronic neuritis, gouty and uric acid diathesis, fattv and 
dilated heart ; in scurvy, haemophilia, purpura, and grave anaemias, 
malarial fever, pyaemia, leukaemia, suddenly suppressed menstruation, 
and the menopause. 

Retinal hemorrhages may occur with or without true inflammation of 
the membrane. The extravasation may be small and confined to the 
retina, or may be large enough to fill the vitreous. Rupture of the 
vessels near the macula or extensive bleeding into the structure of the 
retina seriously interferes with vision, but many small extravasations, 
especially those upon the nasal side or in the periphery, may not be 
noticed by the patient. Hemorrhages which occur in the nerve-fibre 
layer of the retina are flame-shaped, while those into the deeper layers 
are round or oval, fresh ones appearing as dark red blotches ; small 
hemorrhages are rapidly absorbed, leaving whitish patches surrounded 
by pigment, or a simple mass of pigment. If bleeding occurs into the 
vitreous, whitish streaks may remain, which have been given the name 
of retinitis proliferans. The distinction between simple hemorrhage 
into the retina and retinitis with hemorrhages cannot be definitely 
drawn. Most forms of retinitis are attended with effusion of blood 
either from diapedesis or rupture of the vessel wall. Under such con- 



368 THE EYE AND NEKVOUS SYSTEM 

ditions the ophthalmoscope shows numerous small extravasations, espe- 
cially marked at the posterior pole, oedema of the nerve-head, venous 
dilatation and arterial contraction, patches of degeneration, and pig- 
ment changes. In apoplexy of the retina, extravasation takes place 
without apparent preceding inflammation. This causes destruction of 
the retinal elements, and induces inflammation in their immediate 
neighborhood. 

Hemorrhages into the optic nerve unaccompanied by retinal extrava- 
sations are rare. They appear as irregularly outlined dark red clots, 
and when unaccompanied by neuritis are indicative of arteriosclerosis. 

Prognosis. — Hemorrhage into the retina is a grave affection, the 
restoration of vision depending upon the situation of the apoplexy and 
degree of destruction of the retinal elements. Complete restoration of 
the function of rods and cones does not occur. Hemorrhage into the 
retina is a forerunner of cerebral apoplexy, a symptom of brain tumor, 
heart or kidney disease, diabetes, and blood affections. Where found, 
a thorough investigation of the vascular system and abdominal organs 
should be made. 

Treatment. — Although the resorption of the blood is probably as 
active without any treatment as with, yet rest of the body and eyes 
prevents further bleeding. Iodide of potash and soda or mercurials 
should be administered to produce more rapid resorption of the blood 
elements. Dionin locally in 5 to 10 per cent, solutions, or subconjunc- 
tivally, or in powder, is an active lymphagogue and aids resorption. 

Embolism of the Central Artery. — The main stem of the artery 
may be occluded in the optic nerve, or the embolus may lodge in one 
of the branches. The course of the blood is thereby interrupted and 
the retina being deprived of its supply, loses its function. Should the 
clot occupy the entire calibre of the artery, total and sudden blindness 
follows; should it be small enough to pass into a retinal branch, the 
blindness will correspond to that part of the retina supplied by the 
affected vessel (Plate V., A and B) ; should the clot only partially 
block the lumen of the vessel, loss of vision is at first partial, but gener- 
ally becomes complete. In such cases recurrent attacks of temporary 
blindness are liable to occur, usually followed by an atack in which 
there is permanent blindness. A portion of the retina may receive its 
blood-supply from a cilioretinal vessel and thus retain its function in 
a small area of the field. Embolism is usually monocular. 

Causes. — This condition is indicative of cardiac or vascular disease, 
ulcerative endocarditis, valvular disease, particularly atheroma of the 
arteries. In a small proportion of cases the sudden loss of sight in 



PLATE V. 




E F 

A. Partial embolism ; superior branch retinal artery (Haab) ; B. Total embolism central artery in 
nerve ; a cilio-retinal vessel supplies a small area of the retina in which function is preserved (H. V. W.) ; 
C. Thrombosis of central vessels from mumps (H. V. W.) ; D. Results of thrombosis of central vessels : 
same case 6 months later (H. V. W.) ; E. Hemorrhages from retinal vessels ; apoplexy of retina (Magnus) ; 
F. Perivasculitis luetica (Magnus). 



DISEASES OF THE RETINA 369 

chronic nephritis is due to this cause. Syphilis and degeneration of 
the arteries may be suspected. Embolism has been said to occur in 
chorea. 

Ophthalmoscopic Appearances. — These are characteristic ; in complete 
occlusion the arteries carry no blood, and the intima only is visible ; 
the veins and papilla are pale and the retina cedematous ; sometimes 
small hemorrhages are found. A blood-red cherry spot appears in the 
fovea, resembling a small round hemorrhage. As a consequence of the 
ischsemia atrophy of the retinal elements and of the optic nerve ensue. 

Symptoms. — In some cases the ultimate blindness is preceded by 
occasional transitory attacks of loss of sight. In complete obstruction 
of the central artery the loss of vision is immediate and entire, and 
in most cases permanent. In a few instances the clot has been dislodged 
and broken up and absorbed by treatment, securing restoration of func- 
tion. In others where a part of the retina regained its function by 
an anastomosis through the choroidal vessels, or where cilioretinal ves- 
sels existed, a certain degree of restoration may occur. 

Diagnosis. — The sudden monocular blindness, the cherry-red spot 
at the fovea, the diffused retinal oedema, absence of pain and inflamma- 
tion, render the diagnosis positive. 

Prognosis. — If the patient is seen within a few hours or days after 
the attack, treatment may restore vision, though if this does not occur 
within a short period, the retina and nerve will atrophy and the blind- 
ness persist. 

Treatment. — If the case be seen within the first few days, deep mas- 
sage through the closed lids by the thumb or by a mechanical masseur, 
or by forcibly pressing the eye back in the orbit and holding it there 
for a few seconds, may break up or dislodge the embolus ; this con- 
joined with subsequent treatment by large doses of iodide of potash, 
saline cathartics and pilocarpine sweats has succeeded in a number of 
reported instances in securing partial or complete recovery. 

Thrombosis of the Central Vein. — Ophthalmoscopic Appearances. — 
In this condition there is an unusual dilatation and tortuosity of the 
veins, the latter appearing like sausages (Plate V., C), while the 
arteries are contracted to narrow white lines. There are numerous 
hemorrhages and intense papillitis. After a few weeks the hyperemia 
lessens, but atrophy of the retina and nerve ensues. (Plate V., D.) 

Symptoms and Causes. — Vision, although seriously affected from the 
first, may not be entirely absent, though eventually it is lost. The occlu- 
sion of the vein is due to inflammation following phlebitis or endarteritis 
usually as a consequence of microbic infection (Fig. 10). It may occur 
24 



370 



THE EYE AND NEKVOUS SYSTEM 



from erysipelas, abscess of the orbit, purulent meningitis, mumps, or 
infectious blood-diseases. If the phlebitis has its site in a small vein, 




Fig. 10. —Section through optic nerve entrance in a case of thrombosis of the central retinal vein 

occurring in mumps. 



the signs are confined to the corresponding portions of the retina. It 
is usually limited to one eye, but cases have been reported as occurring 
in both. 

(3) INFLAMMATION OF THE RETINA. 

Retinitis may occur alone, but is usually associated with some 
change in the optic nerve producing neuroretinitis, with ultimate atro- 
phy. Inflammation of the retina is announced subjectively by disturb- 
ance of vision, particularly of the central acuity, contraction or limita- 
tion of the field, scotomata, and other field disturbances. Ophthalmo- 
scopically there may be oedema, opacity, hemorrhage, exudation, pig- 
ment changes, and alterations of the vessels of the nerve-head and 
retina. (Plates IV. and V.) 

Visual Disturbances. — The sensibility of the retina may be increased 
or diminished ; in the former instance there is hyperesthesia, the retinal 
elements being so sensitive that subjects see best in subdued light (nyc- 
talopia). In neurasthenia the retina is sometimes acutely sensitive, 
objects being perceived during both day and night that are not ordinarily 
visible to healthy eyes. 

Day-Blindness. — The sensibility of the retina may be so decreased 
by long exposure, as in snow-blindness, that the retina cannot perceive 
objects illuminated by a moderate amount of light. Such disturbances 
may occur also from nutritional changes, as is evinced in retinitis pig- 
mentosa and in hemeralopia (night-blindness). 



DISEASES OF THE RETINA 371 

Photopsia is due to irritation of the retinal cells and nerve-fibres, 
and is an early symptom of retinal disease. Sparks, flashes of light and 
color, which are more pronounced when the eyes are closed or in the 
dark, portend serious disease of the eye, such symptoms being commonly 
met with in the early stages of detachment of the retina. They may 
be occasioned by transient disturbances of the circulation from con- 
stitutional affections and from the exhibition of certain drugs, such 
as santonin, etc. 

Metamorphopsia is the term applied to distortion of an object as a 
result of partial destruction of the rods and cones or obstruction by 
retinal pigment or hemorrhage. Patients with this symptom complain 
of overlapping of words and letters and of straight lines appearing 
curved or broken. 

Varieties of Retinitis Associated With General 
and Nervous Affections. 

Retinitis pigmentosa is a gradual degeneration of the" nerve-cells 
and fibres of both the retina and optic nerve with pigment infiltration 
into the structure of the retina. It is primarily a disease of the retina, 
and in some rare cases the pigment infiltration is absent. The disease 
begins in early life, and is inherited in 33 per cent, of the cases, 70 
per cent of which occur in males. It is sometimes complicated by other 
congenital defects of development. About 25 per cent, of the patients 
are offspring of consanguineous marriages. In these families syphilis 
has been found in most instances. The central visual acuity is preserved 
to a marked extent, though peripheral vision is usually much restricted 
or lost. The ophthalmoscopic appearances are characteristic. The 
pigment cells of the retina are collected into masses, resembling bone 
corpuscles, and are distributed mainly along the blood-vessels, appear- 
ing first near the equator, and are usually more thickly distributed 
at this point. The arteries are diminished in calibre and appear 
as whitish lines ; the veins later undergo similar changes. The 
retina finally atrophies and the optic nerve becomes white and very 
much shrunken. 

Symptoms. — The earliest symptom is night-blindness (hemeralopia), 
the visual acuity being greatly lowered by diminished illumination, due 
to ansesthesia of the retina. Ring scotoma are sometimes present, the 
visual field is greatly concentrically contracted, the central vision re- 
maining moderately acute. Nystagmus is generally present. The 
progress of the affection is slow, and the patients often die of inter- 
current disease before vision is totallv lost. 



372 THE EYE AND NERVOUS SYSTEM 

Treatment. — No restoration of vision can be secured. Strychnine 
injections and galvanism, and lately the high frequency faradic cur- 
rent, are advocated. Nerve tonics, good diet, open-air life, and iodide 
of potash are advised. 

Syphilitic retinitis is usually secondary to chorioiditis, but the acute 
manifestation may first appear in the retina. 

Ophthalmoscopic Appearances. — (Edema of the retina, especially near 
the macula, is first observed. (Plate IV., E.) This is followed by 
serous infiltration, the vessels become tortuous, and are partially con- 
cealed by the exudation. The retina usually retains its normal appear- 
ance towards the periphery, although isolated patches of retinitis may 
occur some distance from the papilla. The swelling may reach a milli- 
metre or more, is whitish in color, and free from blood-vessels. The 
arteries are accompanied by white lines, indicating sclerosis of the 
walls arid diminished calibre. The posterior part of the vitreous is 
infiltrated with a fine dust-like opacity. These particles of opacity are 
separate and distinct from one another, but may later form a membrane, 
due to their coalescence and incomplete absorption. Hemorrhages and 
pigment degeneration only occur after absorption of the oedema and 
the retina has been partially destroyed. A concomitant disseminated 
chorioiditis is likewise to be observed at this time. (Plate IV., E.) 

Pathology. — As a rule, the retinitis is secondary to primary inflam- 
mation of the chorioid, although ophthalmoscopic examination may at 
first reveal no symptom of the latter disease. The first changes are a 
vasculitis, consisting of proliferation of the endothelium, infiltration 
of the walls of the vessels passing later into sclerosis. Subsequently 
infiltration of the retina from pigmentary migration occurs which 
extends mainly along the course of the vessels. Eventually both cho- 
rioid and retina become atrophied and degenerated, and are united in 
places by cicatricial changes. 

Prognosis. — As syphilis is a curable disease and readily amenable 
to treatment, the destructive processes in the retinal and chorioidal ele- 
ments are favorably influenced by prompt antisyphilitic treatment. 
Impairment of vision is to be expected, however, in all cases, as some 
degree of atrophy, not only of the retina and chorioid, but of the optic 
elements, always results. 

Symptoms. — Central vision is diminished by the vitreous opacities 
and retinal oedema, and may be reduced even to the perception of light, 
though ultimate partial recovery is attained. The field may be con- 
tracted, and there are usually central and disseminated scotomata. Pho- 
topsia and metamorphopsia are also present. There is little pain even 



DISEASES OF THE KETIKA 373 

when iritis is present, which is a not infrequent complication, as the 
inflammation in the iris is usually mild and insidious and may not be 
recognized until after the formation of synechias. Though the disease 
is usually accompanied by a true uveitis, there is but little photophobia 
and but few signs of external irritation. 

Treatment. — Energetic antisyphilitic treatment by mercurial inunc- 
tions should be pushed to mild ptyalism ; pilocarpine should be injected 
hypodermically and followed by hot baths ; iodide of potash should be 
administered in increasing and large doses, together with the application 
of hot compresses to the eyes. By these measures the disease may be 
shortened, relapses prevented, and the sight preserved. 

The recurrence of retinitis in syphilis indicates a tertiary form of 
the disease and is an evidence of severe infection. 

Albuminuric Retinitis. — Very rarely in acute inflammation of the 
kidney, but in about 10 per cent, of persons with chronic nephritis, 
pathologic degeneration of the retina and optic nerve, and with this 
changes in the pia mater and other brain structures arises. These in- 
flammations of the retina, optic nerve, and meninges are due to retention 
in the blood of toxic elements consequent upon the disturbed functions 
of the kidney and impairment from deficient nutrition. Transient 
cerebral oedema, with increased vascular tension, which is dependent 
upon ursemia, is found in the late stages of nephritis and in kidney 
disease due to pregnancy. The latter conditions are characterized by 
complete and sudden loss of vision, but are unattended by retinal or 
optic nerve changes,- whereas in the former characteristic nerve and 
retinal lesions are developed. 

Ophthalmoscopic Appearances. — These are so characteristic that they 
should not be confused with changes other than those due to extensive 
oedema of the retina and optic nerve. In the early stages of albuminuric 
retinitis there is oedema and hypersemia of the retina, the edges of the 
nerve are blurred and small hemorrhages occur into the retina. In 
the macular region there are usually small, round, or oval glistening 
white dots and streaks, which slowly increase in size until they form a 
characteristic star-shaped figure. When this appears, the veins become 
tortuous, the optic disk is more swollen, and more hemorrhages occur; 
then irregularly shaped isolated patches appear in places, which later 
coalesce, showing marked degeneration of the retinal elements. The 
hemorrhages, depending upon their location in the retina, are either 
round, when they are situated in the deeper layers, or flame-shaped, 
when they occur into the nerve-fibre layer. The whitish patches and 
streaks are shown by the microscope to be due to fatty degeneration of 



374 THE EYE AND NERVOUS SYSTEM 

the nerve-fibres, which later become hypertrophic and oedematous. The 
inflammation of the retina gradually disappears after a few months 
or a year, and atrophy of the retina and optic nerve ensues. The dis- 
ease is almost always binocular, but the ophthalmoscopic appearances 
vary in the two eyes, depending upon the amount of hemorrhage and 
fatty degeneration and the grade of the optic neuritis. (Plate IV., 
C and D.) It occurs only in adults and is most common in women. 

Diagnosis. — The radiating macular figure is almost characteristic, 
although a very similar condition is seen at times in papillitis occur- 
ring in the course of tumor of the brain. Examination of the urine 
is necessary to establish the diagnosis. 

Prognosis. — The occurrence of retinitis albuminurica is exceedingly 
grave; the duration of life in hospital patients averaging eighteen 
months, though in well cared for private patients it is considerably 
longer. The retinitis of pregnancy and acute nephritis, especially 
in young persons, is not so unfavorable. Induced premature delivery 
of the foetus is usually followed by subsidence of the nephritis and 
a certain degree of recovery of vision, although some atrophy of the 
nerve with loss of central acuity and contraction of the visual field takes 
place, depending upon the amount of actual degeneration in the retina. 
It is said that this form of retinitis is usually of the serous rather than 
of the exudative type. 

Treatment. — Treatment is that of the nephritis, or removal of the 
cause, as in induced abortion for the retinitis of pregnancy. Local 
remedies, with exception of smoked glasses and non-use of the eyes, are 
of no effect. 

Diabetic Retinitis. — The fundus changes resemble those of albu- 
minuric nephritis, although the degeneration is not so marked about 
the macula and the star-shaped figure is not usually present. Hemor- 
rhages are more common; the optic nerve is moderately swollen; the 
arteries contracted ; the veins dilated and tortuous. Small white patches 
of degeneration are usually found about the fovea, but may occur any- 
where in the fundus. Retinitis in diabetes is a comparatively rare 
complication, being much less common than cataract, iritis, other forms 
of uveitis and opacities of the vitreous. 

Anaemic Retinitis. — Changes in ansemia are usually limited to local 
anaemia, but in leucocythsemia and pernicious ansemia there are pro- 
nounced alterations. The arteries may be contracted, the veins dilated 
and tortuous, and hemorrhages and oedema of the retina, with a few 
white patches of exudation with edges colored from extravasations of 
blood, and slight optic neuritis, may be present. (Plate IV., B.) 






DISEASES OF THE OPTIC NERVE 375 



(4) INFLAMMATION OF THE OPTIC NERVE. 

Optic Neuritis (Papillitis, Choked Disk). — The most pronounced 
and important eye symptoms of certain general disorders and many 
forms of disease of the nervous system manifest themselves in an in- 
flammation of the optic nerve and an associated retinitis in which fluid 
and solid exudation fills up the interstices of the fibres of the nerve, 
and even the physiologic excavation. The optic nerve elements are 
swollen, crowded together and distorted, thus elevating the papilla above 
the level of the surrounding retina, and, by pressing upon the ves- 
sels, partly obliterate and conceal them. The veins become occluded, 
giving rise to numerous hemorrhages. If this condition prevails for 
any length of time, atrophy of the nerve elements and the connective 
tissue takes place. 

Optic neuritis may be differentiated into three classes: (lj medul- 
lary or ascending neuritis, or true inflammation of the nerve-tissue ; 
(2) papillitis, or simple swelling of the intra-ocular portion of the 
nerve from irritation or pressure ; ( 3 ) descending neuritis, originating 
from intracranial diseases, affecting the nerve or its sheaths. 

Ascending neuritis commences in the papilla and usually terminates 
in the brain. Descending neuritis commences in the brain, later finding 
its way down the nerve-sheath or nerve elements into the eyeball. Choked 
disk is an arbitrary expression, denoting swelling of the papilla from 
2 D to 6 D, or even more. (The difference between the highest point 
on the disk and that of the surrounding retina in diopters may likewise 
be expressed in millimetres, 2^ D being equivalent to 1 millimetre of 
swelling) . 

Causes. — The causes of optic neuritis are ocular, orbital, intracranial, 
and constitutional. 

Ocular causes include chorioiditis and retinitis, which are, however, 
usually dependent upon some constitutional cause. 

Orbital. — Fracture of the orbit, cellulitis of the orbit, hemorrhage 
into the cellular tissue of the orbit or nerve-sheaths, deformities of the 
skull, affections of the neighboring sinuses, etc., may be enumerated 
under this heading. 

Intracranial. — Optic neuritis may occur in the course of diseases 
of the brain ; these may be enumerated in order of frequency, as fol- 
lows : meningitis, tumors, gummata, sclerosis, tubercle, cysts, and aneu- 
rism. A low grade of retinitis and optic neuritis is seen in some cases 
of epilepsy and general paralysis. In children probably the most fre- 
quent cause of neuritis is tubercular meningitis. Intracranial affections 



376 THE EYE AKD KEKVOUS SYSTEM 

give rise to optic neuritis by direct pressure upon the chiasm, the optic 
nerves, and the tracts ; or by occasioning increase of the ventricular 
and subarachnoid fluids and disturbing the equilibrium of the different 
portions of the brain ; or by disturbances of nutrition through inter- 
ference with the circulation. 

The precise manner in which intracranial growths occasion optic 
neuritis is still disputed. Yon Graefe was of the opinion that the neu- 
ritis was due to a strangulation of the lymphatics of the head of the 
nerve, as a consequence of the increase in intracranial pressure, occa- 
sioning a blocking of the cavernous sinus and of the tributary oph- 
thalmic vein. This theory has been supported by Manz and Schmidt- 
Kimpler, who demonstrated that the fluid displaced by a cerebral tumor 
found its way into the lymph-spaces of the optic nerve, distending the 
intersheath spaces with fluid. Leber and Deutschmann, on the other 
hand, believe that the choked disk is not merely a stasis but claim that 
the presence of the fluid sets up a toxic condition which gives rise to a 
true inflammation of the nerve. Others incline to the view that the 
neuritis is a true descending one, as they have shown that there is an 
increased cellular exudation in the tissues surrounding the cerebral 
tumor, which extends the whole way from the tumor to the nerve and 
along the latter to the eye. In view of these facts, it would appear 
that the element of intracranial and intervaginal pressure plays an 
important part in the production of choked disk, but that the latter, in 
the majority of cases at least, depends upon an active inflammation. 

Choked disk and optic neuritis occur in about 90 per cent, of cases 
of brain tumors, irrespective of their size or location. The absence of 
optic neuritis does not exclude the diagnosis of brain tumor, but its 
presence is a strong confirmatory sign. Tumors of the occipital region 
and the base of the brain are apparently more often accompanied by 
choked disk than those in other portions (Compare Chapter IX). 

Constitutional. — Optic neuritis sometimes occurs in eruptive fevers, 
diphtheria, epidemic influenza, and other contagious diseases; it may 
also be occasioned by albuminuria, especially of the puerperal type, 
diabetes, leucocythemia, toxemia, pernicious anaemia, acute rheumatism 
after exposure to cold, and menstrual affections. 

It is believed by some that in all cases of optic nerve atrophy there 
is first an active, although evanescent, primary inflammation of the optic 
nerve, which is manifest though for a short time upon the optic disk. 
In spinal cord affections a slight degree of neuritis or neuroretinitis is 
a forerunner of optic atrophy. Optic neuritis occurring in the course 
of syphilis is an evidence of the tertiary stage of the disease. 




PLATE VI. 




E F 

Optic Nerve Changes in Brain and Spine Diseases.— A. Atrophy following infantile cerebral 
(Yellowish Gray Neuritic Atrophy) ; B. Atrophy in disease of the optic nerve between bulb and 
chiasm (Atrophy of Macuho Fibres) : C. Atrophy in disease of the chiasm; D. Atrophy in tabes (White 
Spinal Atrophy) ; E. Optic neuritis in basilar meningitis; F. Choked disk in tumor of the cerebrum. 



DISEASES OE THE OPTIC JSTEKVE 377 

Ophthalmoscopic Signs.— The inflammatory process, as observed by 
the ophthalmoscope, may be arbitrarily divided into three stages, which 
pass imperceptibly into each other. 

First Stage. — The border of the papilla is blurred by infiltration 
or swelling; the physiologic excavation is filled in and the disk red- 
dened, as a consequence of relative increase in the size of the blood- 
vessels, whereby fine twigs invisible in health are brought into view, 
and the veins increased in size and tortuosity. The main arteries of 
the disk are contracted, the connective tissue becomes hypertrophied 
and opaque and conceals the disk outlines. The papilla projects for- 
ward, as may be determined by focussing the blood-vessels and its 
surface. (Plate VI., E.) During this stage there are no marked 
changes in the retina excepting dilatation, tortuosity and dark color 
of the veins, and the disease may not advance ophthalmoscopically 
beyond this condition. 

Second Stage. — If the inflammatory process continues in intensity, 
these ophthalmoscopic signs increase. Then the swelling of the nerve 
may become more marked and the disk may project as much as three 
millimetres (7-J D) into the vitreous. (Plate YL, F.) Its outlines 
are hidden by exudation and hemorrhage, and the swelling over- 
lapping in all directions apparently doubles the size of the papilla. 
Its position is thus only determined by the entrance of the blood-vessels 
which are obscured and interrupted in places and difficult to follow. 
The veins are enormously dilated, tortuous and dark in color, appar- 
ently outnumbering the arteries, which are proportionately reduced in 
number and calibre and carry little blood, showing in places as simple 
white lines. Small flame-shaped hemorrhages appear near the disk. 
The retina in the neighborhood is cedematous, and its fibre layer hyper- 
trophied, showing radiating white lines. This stage varies in duration 
from a few weeks to several months, gradually passing into the stage 
of degeneration. 

Third Stage. — Absorption of the blood and exudation now takes 
place, the swelling of the nerve-head declines, the periphery contracts, 
the exudative material is absorbed or transformed into connective tissue, 
the veins are less tortuous, and the arteries smaller. The papilla be- 
comes a dirty grayish white, and a firm, organized exudation fills in 
the physiologic excavation, completely hiding the lamina. The nerve 
passes ultimately into complete atrophy. (Plate VL, A.) 

Symptoms. — Visual Acuity. — The vision is usually but little affected 
at first, and thus few patients are seen until the second stage ensues, 
when the vision is usually somewhat reduced, the reduction becoming 



378 



THE EYE AKD NERVOUS SYSTEM 



gradually worse during the continuance of the disease. Large hemor- 
rhages in the region of the macula may, however, cause more or less 
sudden blindness. Exceptionally there is no great deterioration of vision 
until the third stage, and thus a considerable degree of papillitis may 
exist with normal visual acuity. Hemorrhage or exudation into the 
macular region and other parts of the retina, permanently diminish 
the function of the retinal cells and fibres by pressure, and later by 
cicatricial tissue formation and contraction. As a rule, vision dimin- 
ishes to light perception or ultimate blindness in the cases which outlive 
the ocular affection. 

Visual Field. — The changes in the visual field are important in 
localization of the cerebral lesion. The most common defects are 



,- Eye 




Fig. 11.— Enlarged blind spot with paracentral 



Fig. 12.— Pericentral absolute and Ring sco- 



absolute and relative scotomata in optic neuritis toma for colors, in syphilis, due to probable gumma 
irom brain tumor. of optic nerve. 



peripheral contraction, enlarged blind spot, or central scotomata. The 
former is characteristic of uniform pressure upon the nerve by fluids 
or solids in or external to its sheath, while the latter indicates axial 
neuritis with loss of function of the central nerve-fibres. Sector-like 
defects involving definite portion of the visual field, such as hemian- 
opsia, which may be, — i.e., homonymous (nasal or temporal), heteron- 
ymous (or lateral) tetr anopsia (loss of a quarter of the field), and 
smaller defects occur, which are symptoms of descending optic neuritis, 
due to lesions of the chiasm, tract or deep centres. These are usually 
binocular and symmetrical. Monocular defects arise from pressure 
upon the nerve in the orbital canal or orbit, or upon a portion of the 
chiasm or upon small areas of the occipital lobes. 

The color fields diminish more rapidly than the perception for white 



DISEASES OF THE OPTIC NERVE 



379 



and form, though there is usually peripheral contraction for all colors. 
As the disease progresses, green is first lost, then red, and finally blue. 
The loss of the blue field is contemporaneous with marked contraction 
of the form field and indicates advancing atrophy. As the scotomata 
and sector-like defects in the visual field, which occur from intra-ocular 
disease secondary to changes in the retina, frequently resemble those 



L.E. 



B.E. 




180 [— 80-70-60-50-40-30- 



Fig. 13.— Temporal hemianopia and peripheral contraction from tumor of optic chiasm, causing 

bilateral choked disk. 



from changes in the cerebral centres, it is necessary to rely upon oph- 
thalmoscopic examination to establish the diagnosis. (See Chapter IV.) 
Prognosis. — Optic neuritis of all grades is grave, because the intra- 
cranial and constitutional diseases of which it is a lesion involve essen- 
tial organs of the body. An unfavorable prognosis as to sight may 
usually be given, with the exception of cases of toxic amblyopia, in 
which full recovery often occurs after removal of the cause. Atrophy 
invariably follows the inflammation, and as the degeneration of the 
nerve progresses, vision diminishes. The atrophy generally advances 
more rapidly in one nerve than the other, so that some useful vision 
may be preserved in one eye even after the other has become blind. 
As regards life, the occurrence of an optic neuritis often indicates 
grave cerebral involvement, and when it is concomitant with retinitis 
or advanced renal or vascular lesions, its occurrence is usually prog- 
nostic of a lethal ending within a few weeks to two or three years, at 
most, depending upon the gravity of the central or constitutional lesion. 



380 THE EYE AND NERVOUS SYSTEM 

Treatment. — The treatment depends upon the cause, for which a com- 
plete physical examination is essential. Discovery of albumin, casts, 
or sugar in the urine, determines the line of treatment, which, as a 
rule, is to be given by the family physician. If the case is found to 
be of cerebral origin, the oculist may be seen only in consultation. 
Syphilitic cases should be combated by energetic mercurial inunctions, 
large doses of iodide of potash, pilocarpine sweats. The rheumatic, 
albuminuric, and toxic types are likewise benefited by pilocarpine. 
Acute forms, where exposure to cold has been followed by neuritis and 
loss of vision, are helped by blood-letting and diaphoresis. Salicylate 
of soda in doses corresponding to the weight of the individual, — i.e., 
1 grain for each pound of weight a day, — are of service. Regulation of 
the menses is an important factor in women. Ethmoid and sphenoid 
disease should be searched for and properly treated. 

Acute Retrobulbar Neuritis. — The optic nerve-fibres may be affected 
primarily by disease posterior to the eyeball from injury or disease 
of the orbit and of the accessory cavities, or by rheumatism, inhalation 
or absorption of toxic substances, such as methyl alcohol or quinine, 
or by influenza, acute alcoholism, and autotoxaemia. 

Pathology. — The function of the nerve is rapidly diminished by 
interstitial inflammation of the nerve-fibres, or by pressure upon the 
nerve or vessels. In some cases the inflammation subsides, as the exuda- 
tion is absorbed or the pressure removed. In others, atrophy ensues 
with more or less complete loss of vision. 

Symptoms. — There is rapid loss of visual acuity, perception of light 
in some cases being lost in a few hours or days. The visual field may 
be peripherally contracted, but, as a rule, there is a central scotoma 
which increases in size until only a small rim of peripheral field re- 
mains. The central scotoma is at first only for color, — particularly for 
red and green. Partial recovery occurs, followed later by further loss 
of vision. Weakness or paralysis of the facial or other nerves may 
accompany or precede the optic nerve affection. 

Ophthalmoscopic Appearances. — The ophthalmoscope shows the nerve- 
head to be hypersemic with discolored surface and obscured edges ; the 
arteries are contracted, and the veins dilated, and in rare cases there 
are a few small hemorrhages. 

Prognosis. — Recovery of useful vision follows early energetic treat- 
ment, though cases usually go on to more or less complete blindness. 

Treatment. — Removal of the cause with local blood-letting, large 
doses of salicylates in rheumatic cases, strychnine hypodermically in 
increasing doses, iodide of potassium, and mercurial inunctions are indi- 






DISEASES OF THE OPTIC XERVE 381 

cated. Pilocarpine sweats eliminate 23oison and assist the action of the 
internal remedies. 

Chronic Retrobulbar Neuritis (Toxic Amblyopia). — The optic 
nerve may degenerate from the constant absorption of poisons, notably 
large quantities of tobacco or alcohol, or, as is usually the case, their 
combined action. Xumerous other poisons, — i.e., lead, quinine, the 
methyl compounds, salicylic acid, napthalin, nitrobenzol, — will pro- 
duce a similar inflammation. Toxic amblyopia may also arise in the 
course of some constitutional diseases, such as diabetes, and one of the 
theories of choked disk is that it is developed by a local toxemia arising 
in the brain. 

Pathology. — The disease consists in a true axial medullary neuritis, 
possibly secondary to degeneration of the -macular ganglion cells, al- 
though it is more probable that it arises in the bundle of the nerve-fibres 



Right „ 
120' 




Fig. 14.— Toxic amblyopia ; small absolute central scotoma ; larger central scotoma for red 
and green; contracted field. 



supplying the macular region. It is a gradual degenerative process and 
which, unless checked, encroaches upon the other fibres adjoining the 
papillomacular bundle until the entire nerve becomes atrophic. 

Symptoms. — The impairment of vision is gradual, and consists in 
blurring of sight, which is not relieved by glasses. The pupillary reflex 
may be disorganized so that the pupil dilates with the accommodation. 
The visual acuity varies from slight derangement to entire loss of cen- 
tral acuity, depending upon the amount of central scotoma. As a rule, 
there is more disturbance for color than for form, the characteristic 
signs of the disease being central scotoma for colors, more particularly 
for green and red. Later in the disease the scotoma, which was at first 



382 THE EYE AND NERVOUS SYSTEM 

relative, becomes absolute, increases in size, and finally the peripheral 
field becomes concentrically contracted. 

Ophthalmoscopic Appearances. — As a rule nothing distinctive can be 
seen in commencing cases, with the exception that the outline of the 
nerve is not so sharp and clear as in the normal subject (Plate V., B.), 
but in advanced cases the nerve-head is usually pale in the lower tem- 
poral quadrant, — i.e., in the region occupied by the macular fibres. 

Prognosis. — Prompt treatment preserves the visual acuity, and in 
favorable cases vision may be entirely restored, the amount depending 
upon the degree of destruction of the nerve-fibres. 

Treatment. — Removal of the cause. If this be alcohol or tobacco or 
both, the patient may have to receive special general treatment, which 
can usually only be properly carried out away from his usual surround- 
ings, in a sanitarium or hospital. Increasing doses of strychnine, hypo- 
dermically, for courses of several weeks, with intermissions of a week 
or two, may restore much of the lost vision. Nitroglycerin and nitrite 
of amyl may be used. If there be exudation into the nerve, the iodides 
are of value. In anaemia, the employment of iron and other tonics, 
nourishing diet, exercise out of doors, early hours, and plenty of sleep, 
are necessary. Galvanic electricity and the high frequency faradic cur- 
rent seem to stimulate the retinal elements to renewed activity. 

(5) OPTIC NERVE ATROPHY. 

Atrophy of the optic nerve may be primary or secondary, the types 
being readily distinguished by the appearance of the optic disk. 

Primary Atrophy.- — Atrophy not preceded by pronounced papillitis 
or neuroretinitis is, in the majority of cases, a symptom of spinal cord 
disease, usually of sclerosis of the posterior columns (locomotor ataxia). 
It occurs less frequently in disseminated sclerosis, lateral sclerosis, epi- 
lepsy, and general paralysis. Toxic amblyopia, both acute and chronic, 
the specific fevers, diabetes, and arteriosclerosis also lead to atrophy of 
the medullary fibres. There is likewise an hereditary form following 
retrobulbar neuritis. It is a mooted question whether or not oedema 
of the nerve is not always present and capable of being observed oph- 
thalmoscopically in the early stages. Where it is concomitant with 
spinal disease the structural changes in the optic nerve may apparently 
precede the spinal symptoms for months or years, and thus the dimin- 
ishing of the visual function may be the first symptom of the general 
affection. (See Chapter XII.) 

Anatomical Changes in Primary Nerve Atrophy. — Ophthalmoscopic 
Appearances. — The papilla gradually assumes a chalky- white appear- 



CH P G Z 



Z G P CI 



j ^ 






u 




Fig. 15.— Sections through the optic nerve. A, normal nerve ; B, papillitis ; 
C, optic atrophy. (Reproduced from Elschnig.) 



DISEASES OF THE OPTIC NERVE 



383 



ance or it may acquire a bluish-green tinge. As the atrophy progresses, 
contraction of the nerve-fibre bundles and their surrounding elastic 
tissue takes place, causing cupping of the nerve. The cupping is grad- 
ual and occupies the surface of the nerve. All the blood-vessels become 
contracted and the arteries may be entirely obliterated, being replaced 
by white streaks, especially when the disease is due to arteriosclerosis. 
This process causes first a flattening of the central nerve-fibres with 
circumscribed atrophy of the marginal or central nerve-fibre bundles, 
and the changes in the papilla are always preceded by failure of vision 
and defects in the visual field. Atrophy of the retina follows that of 
the nerve. 

Microscopic Appearances. — This is not an ascending atrophic pro- 
cess, and the nerve does not become as thin and attenuated as in the 
neuritic inflammation. On section, the septa are found to be well pre- 
served, and the fibres transformed into fine fibrillar which stain with 
carmine. The blood-vessel walls are sclerosed, but the septa only become 
thickened quite late in the disease. Some of the nerve-fibres remain 
healthy, and cases may escape the sclerosis entirely. The surface fibres 
are the first to degenerate and this may show in the visual field as 
peripheral alterations. (Fig. 15, A. B. C.) 

Subjective Signs. — The acuity of vision depends upon the degree of 
degeneration of the nerve-fibres. The first symptoms are diminution 



Eight 




Fig. 16.— Optic nerve atrophy in tabes dorsalis. 



in the light sense and contraction in the color fields; the patient de- 
mands strong light and has difficulty in getting about in the dusk or 
dark. The light sense may be tested by determining the acuity of vision 
in different degrees of illumination, or by the Forster photometer. 



384 



THE EYE AND KEKVOUS SYSTEM 



Usually the contraction of the color fields is greater than that for white, 
and a large white field may remain when the color vision is entirely 
gone (Fig. 16). 

Prognosis: — The disease terminates in partial or complete blindness. 

Treatment. — Treatment is that of the constitutional or cerebral dis- 
ease. Electricity is much used, but is of little avail. Nitroglycerin 
and nitrite of amyl and hypodermic injections of strychnine temporarily 
improve vision. Administration of the iodides of mercury, potash, soda, 
and strontium, and the bromides and iodides of gold, mercury, and 
arsenic, and sweat baths are recommended. 



B.E. 




0-7,0-80— 



300 240 3 

270" 270° 

Fig. 17.— Nasal hemianopia. (After Veasey.) 



Secondary, Ascending, Consecutive, or Neuritic Atrophy. — This 

condition is the result of inflammation of the orbital end of the optic 
nerve following retinitis, neuroretinitis, choked disk, or chorioretinitis. 
The inflammation may furthermore be induced by intracranial lesions, 
tumors, syphilis, encephalitis, meningitis, or distention of the third 
ventricle pressing upon the chiasm, or may result from retrobulbar 
neuritis, injury, oedema, or pressure on the optic nerve at the optic 
foramen, from alcoholism, from the manifestations of syphilis, tuber- 
culosis, nephritis, etc. 

Ophthalmoscopic Appearances. — The changes in the optic nerve are 
somewhat different from 'those of primary or spinal atrophy. Thus the 
nerve-head is usually of a dirty-gray color, is not cupped, and the edges 
are not so well defined as those of spinal atrophy. (Plate VI., A.) 



DISEASES OF THE OPTIC KEEVE 



385 



Microscopic examination demonstrates that this is only a strictly 
ascending process when following purely intra-ocnlar inflammations, 
as chorioretinitis, for in most cases anatomic examination shows that 



L.E. 



E.E 




180 k-8O-oO-60- 



Fig. 18.— Homonymous hemianopia (lateral) with external ophthalmoplegia occurring in disseminated 

sclerosis with nuclear paralysis. 



choked disk or optic neuritis involves the nerve behind the globe, even 
as far back as the optic chiasm and intracranial divisions. 

The subjective signs are much the same as in spinal atrophy. There 
is a loss of vision and contraction of the visual field, the various changes 
in the field depending upon the intracranial lesion or the amount of 
retina that has deteriorated as a result of the intra-ocular disease. 

Treatment. — Therapeutics may check the advance of the atrophy and 
postpone the inevitable blindness, as has been noted in discussing pri- 
mary atrophy. 

Hereditary optic-nerve atrophy is a sufficiently peculiar form to 
demand separate description. Subjects of this form of atrophy are 
usually affected at puberty or early manhood. As a rule, their skulls 
are tower-shaped, and belong to the so-called scapho- and dolicho-cephalic 
varieties. The sphenoid bone is not fully developed until rather late 
in life ; it contains the optic foramen, and any irregularity in its growth 
may cause in this type of skull a lateral pressure which occasions the 
peculiar ingrowing visual field defect which invades the field of fixation 
and ultimates in the loss of central vision. (See Chapter XIV.) 

In some cases the central scotoma is not accompanied by marked 
25 



386 



THE EYE AND NERVOUS SYSTEM 



narrowing. This may be explained by the fact that the axial fibres 
immediately behind the bulb are quite lateral, and may continue so to 
the foramen. 

L. E. R. jE. 



I8O-81 




80— 



Fig. 19.— Hereditary optic-nerve atrophy, showing development of central absolute and relative 

scotomata. 

Cases of this disease usually occur in young males and are trans- 
mitted from one generation to another through the females, who escape. 

Treatment is the same as for other forms of atrophy, but is practi- 
cally unavailing. 



CHAPTEE IX. 

TUMOES AND OTHEE LESIONS OF THE BEAIN, ESPECIALLY 
THOSE IN EELATION TO THE VISUAL APPAEATUS AND 
OCULAE NEEYES. 

By WILLIAM G. SPILLER, M. D. 
GENERAL SYMPTOMS OF TUMOR. 

So much has been written on encephalic tumors, both by American 
and foreign writers, that it has seemed well in this chapter to pay more 
attention to new growths that are of special interest to ophthalmologists. 
American writers have made notable contributions to this subject. 
Tumors not connected with the visual system will therefore be treated 
rather briefly in this chapter. 

Tumors of the brain may cause symptoms that are not indicative of 
the position of the growth but merely show that something abnormal is 
developing within the cranial cavity producing an increase of intra- 
cranial pressure. The most important of these symptoms are headache, 
often persistent and with exacerbations, nausea and vomiting, general 
convulsions, vertigo, and choked disks. They do not imply necessarily 
that a tumor is growing within the brain, and they may be caused by 
meningitis, abscess, or other lesions. There are in most cases of tumor 
certain signs and symptoms that give some clue to the location of the 
cerebral growth ; and the determination of these is of great importance. 
Choked disk in itself does not show whether a tumor is in the cerebrum 
or cerebellum. Marcus Gunn's opinion that unilateral, or prepon- 
deratingly unilateral optic neuritis is in favor of the tumor being on 
the same side as that on which the neuritis is more intense can not be 
accepted for all cases. Purves Stewart, for example, says he has seen 
a case of large frontal tumor in which the optic neuritis was both earlier 
and more intense on the opposite side from the lesion. This is an inter- 
esting observation, because we might expect that if unilateral optic 
neuritis were indicative of the side on which the tumor is situated, it 
would be so especially when the tumor is in the frontal lobe. 

LESIONS OF THE OCCIPITAL LOBE. 

There are certain signs and symptoms pointing to tumor of the 
occipital lobe, and yet they are not pathognomonic. Early developing 

387 



388 THE EYE AKD NERVOUS SYSTEM 

hemianopsia or hemiachromatopsia, sensations of light, visual hallucina- 
tions in the blind fields, with absence of Wernicke's hemianopic pu- 
pillary sign and with the general symptoms of brain tumor are indica- 
tive of a growth in the occipital lobe. In some cases the symptoms of 
cerebellar tumor are added and are caused by pressure upon the cere- 
bellum. (For remarks on optic neuritis see Chapter VIII, p. 375.) 

Hallucinations of Sight. — Ferrier and Jackson are said to have been 
the first to observe photopsia in disease of the occipital lobe. The visual 
hallucinations usually appear in the blind fields. Wilbrandt and 
Henschen believe that these visual hallucinations occur only with sub- 
cortical hemianopsia, and not when the cortical visual centre alone is 
disturbed. (See also Chapter IV, p. 174.) 

Harris, in referring to Swanzy's view that hallucinations of sight in 
the blind fields are possible in lesions of the optic radiations but not in 
hemianopsia the result of cortical damage, says that the reverse seems 
to be more common. The only case with necropsy supporting Swanzy's 
view is that reported by de Schweinitz. Harris refers to a number of 
cases of visual hallucination with hemianopsia but almost all were 
without necropsy. 

Oppenheim mentions that visual hallucinations and illusions may 
be caused by intraocular lesions, and refers to Uhthofx*. This phe- 
nomenon I also have observed. I recall a patient under the care of Dr. 
C K. Mills whom I saw with him. The man had hallucinations of sight, 
and saw various objects, especially horses, as a symptom of hemorrhage' 
into the retina. 

L. Bruns believes that visual irritation symptoms preceding or ac- 
companying hemianopsia are of value as indicating a location of the 
lesion in the occipital lobe. These symptoms may be spots or lines of 
light before the eyes, or hallucinations of sight. 

In a case observed by Byrom Bramwell 1 in which numerous tumors 
were found in the brain, and the occipital lobes and each optic thalamus 
were implicated by the tumors, the patient had frequent paroxysmal 
attacks lasting about five minutes at a time, with headache, flushing, and 
flashes of light in the eyes. The light was especially bright in the right 
eye and had the brilliancy of electricity, and varied in size from a button 
to a large globe. During these paroxysms the patient was completely 
blind. Hemianopsia did not appear to be present after an attack. Bram- 
well attributes these flashes of light in the right eye to irritation of the 
left half-vision centre. It is well to remember, however, that each optic 

1 Byrom Bramwell, Brain, 1899, vol. xxii. p. 41. 



TUMORS AED OTHER BRAD* LESIONS 389 

thalamus was the seat of tumor, and it is possible that the irritation 
symptoms were caused by the implication of these bodies ; such a com- 
jilication was not present in another case observed by him. 

In this second case the cicatrix of an old syphilitic gumma was 
found on the outer surface of the posterior end of the left occipital lobe. 
The patient, a man, complained of severe headache confined to the back 
and top of the left side of the head. He had peculiar sensory (visual) 
Jacksonian epilepsy. The attacks occurred on an average about once 
a year, and each attack, consisting of numerous paroxysms, usually 
lasted three or four weeks. The paroxysms occurred every five or ten 
minutes, and lasted from half a minute to three minutes. They con- 
sisted of flashes of light in the right eye, and the light was said to be 
red and white. Eor some days after the attack the patient was unable 
to see to the right side (right-sided homonymous hemianopsia). 

Jolly has shown that scotoma scintillans may result from a disturb- 
ance of the visual tracts, and more recently A. Pick 2 has demonstrated 
by the report of four clinical cases that hallucinations of sight in the 
hemianopic fields may be caused in the same way. The first of his cases 
shows that a focus of softening probably existed in the left angular 
gyrus. Important symptoms in this case were paraphasia and para- 
graphia and persistent right lateral hemianopsia. At least on one occa- 
sion everything in the right side of the fields of vision was of a reddish 
color and changed later to green. Eor about half an hour a dog was 
seen by the patient walking along with him at his right side. This was 
merely a visual hallucination. 

It seems that hallucinations of sight may be caused by disturbance 
of any part of the visual system from the eyeball to the cortical layer of 
the occipital lobe. 

Pick has observed bitemporal scotoma scintillans, and he attributes 
this phenomenon to a lesion of the chiasm. 

According to Uhthoff, 3 disease of the retrobulbar and intracranial 
optic pathways relatively seldom causes visual hallucinations, but it did 
in one of his cases and he refers to some others in the literature ( Chris- 
tian, de Schweinitz). 

Harris reports a case of partial hemianopsia in which a peripheral 
zone of the left lower quadrant remained intact. The lesion was be- 
lieved to be a softening of the right cuneus. The patient had frequent 
attacks in which he saw red and green lights always to the left in the 



2 A. Pick, Amer. Journ. of the Med. Sci., January, 1904, p. 82. 

3 Uhthoff, Monatsschrift fur Psychiatrie und Neurologie, vol. v. 



390 THE EYE AND NERVOUS SYSTEM 

blind field, and they appeared to be moving in both directions, from the 
periphery towards the centre and in the reverse direction. 

I know of a case in which the diagnosis of fracture of the base of 
the skull was made. The patient at times had visual and olfactory 
hallucinations and saw his bed and other objects move about him and 
turn upside down. 

In the case reported by de Schweinitz the patient had visions of 
chairs, tables, and other articles of furniture, not really present, in the 
fields which afterward were obliterated. Left lateral hemianopsia de- 
veloped, the dividing line passing in advance of the fixation-point. The 
patient was unable to say whether the visual hallucinations disappeared 
immediately after the hemianopsia set in, or whether they remained for 
some time in the dark half-fields. At the necropsy made by Dercum 
gummatous infiltration at the base of the brain pressing upon the right 
optic tract was found. De Schweinitz 4 says he was not aware that any 
lesion of the occipital lobe was present, and it was not probable from 
the symptoms that one would be found. 

Bregman 5 has described green and violet vision occurring in tabes 
dorsalis with optic atrophy, and probably resulting from the irritation 
of the optic nerves. Alter observed a case of paretic dementia in which 
attacks of green vision, lasting one or two hours, occurred, and occasion- 
ally were followed by achromatopsia. Objects were seen in various 
shades of green. It seems probable that these peculiar visual disturb- 
ances in which objects appear red or green may be caused by irritation 
of any part of the visual system, and it is not mere chance that dictates 
the colors seen. The fibres conveying sensation of red and green, if such 
fibres exist, seem to be chiefly affected in optic atrophy, and therefore 
from irritation sensations of light might be more likely to appear as 
red and green vision. The violet seen by Bregman' s patient contained 
red. We should, therefore, from a study of the cases mentioned, be 
very careful before assuming that subjective disturbances of color vision 
indicate a lesion of the occipital lobe. 

I am inclined to think that inverted vision, or mirror vision, may be 
caused by lesions of the occipital lobes, but much study is needed in 
regard to this phenomenon. 

Alexia and Optic Aphasia. — More valuable are alexia and optic aphasia 
as signs of a lesion of the left occipital lobe. These signs are explained 
by L. Bruns as the result of a lesion of the association tracts between 



4 De Schweinitz, New York Med. Journ., May 2, 1891, p. 514. 
6 Bregman, Deutsche Zeitschrift fur Nervenheilkunde, vol. xxvi., Nos. 4 to 6, 
p. 525. 



TUMOKS AND OTHEK BRAIN LESIONS 391 

both occipital lobes and tbe left first temporal convolution at a point 
where these tracts are near together in the white matter of the left 
• occipital lobe. This explanation is by no means generally accepted. 
The symptom-complex is not always typical, but Bruns says it has been 
fairly typical in a number of cases, and was so in one that he himself 
observed. Mind-blindness is present to some extent in association with 
the optic aphasia, but lesions of both occipital lobes seem to be requisite 
for this sign to be pronounced. The reader is referred to the chapter 
by Dr. C. K. Mills on the " Psychology of the Visual Act and the Eocal 
Diseases of the Visual Cortex" for a fuller discussion of achromatopsia, 
mind-blindness, optic aphasia, word-blindness, alexia, and allied .sub- 
jects. 

Transitory amaurosis sometimes occurs with tumors of the occipital 
lobe (Bruns), but according to Hirschberg it has little localizing value, 
as has also conjugate deviation of the eyes, which according to Oppen- 
heim occurs sometimes with occipital lobe tumors. There may be a 
centre for associated ocular movements in the parieto-occipital lobe as 
well as in the frontal lobe. 

Hemianopsia. — The most valuable localizing sign of tumor of the 
occipital lobe is homonymous lateral hemianopsia. This by itself is not 
sufficient, inasmuch as it may be caused by a lesion of the visual path- 
ways anywhere between the chiasm and the visual cortex of the occipital 
lobe. According to Oppenheim, Bernhardt in fifteen cases of tumor of 
the occipital lobe found hemianopsia in only two cases. An examination 
of the more recent reports has shown that rarely hemianopsia is absent 
in cases of tumor of the occipital lobe. 

It has been supposed by some investigators that if the hemianopsia 
is caused by a lesion of the visual cortex the patient will be ignorant of 
his half -blindness until it is pointed out ("vision nulle" of Dufour), 
whereas this will not be the case if the hemianopsia is caused by a sub- 
cortical lesion. This distinction is unreliable. Harris has examined 
for this sign several times without any satisfactory results, and in one 
case in which the lesion was in the posterior part of the internal capsule, 
the patient was not aware of any defect of vision, and could with diffi- 
culty be persuaded that considerable portions of his visual fields were 
lost. (See Chapter IV, p. 167.) 

It seems strange that hemianopsia could be overlooked, and yet a 
case has been mentioned to me by Frankl-Hochwart, in which the 
sign was discovered after it was noticed that the patient, then in a large 
hospital, habitually ate only the food on one half of his plate. It was 
found on examination that he saw only one half of his plate. 



392 THE EYE AKD KEKVOUS SYSTEM 

Hemiachromatopsia. — Hemiacliromatopsia has been considered by 
some as the result of lesion of certain special centres or fibres in the 
visual system. Ward A. Holden refers to a considerable number of 
cases in which, with a normal field for large white test-objects, there 
was hemianopsia for colors. Most of the older writers agree that there 
must be special separate cortical centres for the perception of form, light, 
and color, and that in the cases under consideration, the cortical color 
centre was exclusively involved. This supposition was defended par- 
ticularly by Samelsohn, Landolt, and Wilbrand. Of late there has been 
a tendency to depart from this teaching, and Ole Bull and Otto Dahms 
do not accept it. Some observations of his own have convinced Holden 
that the supposition of there being a lesion of some special color centre 
in these cases is wrong. His views are as follows : 

" A slight interference in conduction of any of the fibres of the 
visual tract, leads to an inability to recognize green or even red, or to dis- 
tinguish slight differences in luminous intensity. A more marked dis- 
turbance in conduction leads to the inability to recognize blue, or to 
distinguish quite marked differences in luminous intensity. A greater 
interference with conduction prevents the distinguishing of white from 
black, and with complete interference with conduction even perception 
of light is lost. 

" Thus the recognition of color varies with the light sense, and the 
assumption of the involvement of a particular cortical color centre in 
cases of hemiachromatopsia is not only unnecessary, but is palpably 
erroneous." 6 

De Schweinitz 7 expresses the opinion that homonymous hemiachro- 
matopsia probably is caused by a cortical lesion of less intensity than 
one which produces absolute hemianopsia. In a case studied by him 
at the onset the hemianopsia was absolute; later light sense and form 
sense returned. The obliteration of the color sense remained, although 
in all other respects the patient had recovered. Non-cortical lesions, he 
holds, may also produce hemiachromatopsia. 

An interesting case recorded by Harris shows that a lesion in the 
neighborhood of the internal capsule may cause hemiachromatopsia, and 
that the lesion need not be cortical to produce this sign. In his case 
there were almost complete left hemiplegia, partial left hemianesthesia, 
and complete loss of all color perception, even of large area, in the left 
lower quadrant for each eye; small objects, as the head of a pin or a 

6 Holden, Archives of Ophthalmology, vol. xxiv. p. 447. 

7 Diseases of the Eye, p. 559. 



TUMORS AKD OTHER BRAES' LESIOXS 393 

disk of white paper 1 centimetre in diameter, were also invisible, and 
the patient could not distinguish between one and two fingers held up 
in the same area, though he could readily do so in each of the remaining 
quadrants. The cuneus was found to be normal on each side, but there 
was an area of softening in the right internal capsule. 

Harris quotes from Swanzy's " Handbook of the Diseases of the Eye 
and their Treatment :" " It is now generally believed that relative 
hemianopsia (e.g., color hemianopsia alone) is the result of a lesion of 
less intensity than that which causes absolute hemianopsia . . . and 
that the color sense is more easily affected by disease than the form or 
light sense, and that, too, irrespective of the position of the lesion in the 
visual path.' 7 

Hemianopic Pupillary Reaction Sign. — The absence of the hemianopic 
pupillary rigidity of Wernicke points rather to a tumor of the occipital 
lobe when a lesion of the visual pathways is probable, as shown by 
lateral homonymous hemianopsia, but the sign is difficult to obtain and 
the danger of error is great. I believe, however, that I have seen this 
sign positively demonstrated in a few cases. It consists in contraction 
of the iris to light only when the halves of the retinae still preserving 
visual function are illuminated. As there is no light reflex from illu- 
mination of the blind half of each retina when this sign is present, the 
lesion must be somewhere within the pupillary reflex arc, therefore in 
or below the primary visual centres (external geniculate body, pulvinar 
of the optic thalamus, and anterior colliculi of the corpora quadri- 
gemina). This sign cannot be caused by a lesion above the primary 
visual centres, in the optic radiations or visual cortex, although there 
may be hemianopsia from such a lesion. (See Chapter IV, p. 170, and 
Chapter VI, p. 318.) 

Paralysis of Cranial Nerves and Cerebellar Ataxia as Signs of Occipital 
Tumor. — When paralysis of cranial nerves occurs in association with 
homonymous lateral hemianopsia it is in favor of a growth below the 
occipital lobe, and yet it is not absolute proof of a basal lesion, especially 
when the paralysis implicates the sixth nerve of either side. This nerve 
has so long a course within the cranium that it may be injured by in- 
creased intracranial pressure. Oppenheim believes it is possible for the 
nerves of the £>osterior cranial fossa to be affected by the pressure of an 
occipital tumor. 

Jacksonian motor epilepsy cannot be regarded as a common sign of 
tumor of the occipital lobe. Cerebellar ataxia has been observed, and has 
been explained as the result of pressure upon the cerebellum. s The 



L. Brans, Die Geschwiilste des Nervensystems. S. Karger, Berlin, 1S9" 



394 THE EYE AKD NERVOUS SYSTEM 

tentorium usually is sufficient to prevent j)ressure upon the cerebellum, 
but in a case of internal hydrocephalus in the service of Dr. Mills, 
caused by closure of the aqueduct of Sylvius, which I had the oppor- 
tunity to study and have reported, pressure on the cerebellum from the 
distended posterior horns of the lateral ventricles seemed to afford a 
satisfactory explanation of the ataxia. This case is as follows : A boy, 
aged nineteen years at the time of death, had had some headache once 
or twice a week, with vomiting, since childhood ; but about six months 
before death the headache became severe, and cerebellar gait was ob- 
served, and he staggered like a drunken man. The station with feet 




Fig. 1.— Almost complete closure of the aqueduct of Sylvius, only the extreme lower end re- 
maining patulous, causing internal hydrocephalus, and symptoms of cerebellar tumor. The fourth 
ventricle was normal in size. 

apart was good, but with feet together was poor, and the sway was not 
increased by closure of the eyelids. The gait was so unsteady that at 
times he had to catch hold of the bed to keep from falling. He was 
occasionally dizzy when lying on his back, and the dizziness was re- 
lieved by turning on his side. Slight exophthalmos was observed. Dis- 
tinct optic neuritis was present in each eye. The patellar reflexes were 
exaggerated. The Babinski reflex was absent on each side. All venereal 
disease was denied. There was certainly sufficient here to suggest a 
cerebellar lesion, but none existed. 



TUMOKS AND OTHER BRAIJNT LESIONS 395 

On removing the skullcap, which was extremely thin, the brain and 
its membranes bulged outward. On the internal surface of the skullcap 
the moulding of the convolutions of the brain could be distinctly made 
out. The third and lateral ventricles were distended with clear fluid, 
and the aqueduct of Sylvius was closed by proliferated neuroglia, while 
the fourth ventricle was of normal size. In microscopical sections a 
very small opening where the aqueduct should be, was found, but dur- 
ing life this may have been closed, and it is doubtful whether it ex- 
tended all the way from the third to the fourth ventricle. 

In a case of echinococcus cysts of the left occipital lobe reported by 
Mingazzini, lateral homonymous hemianopsia was not mentioned by 
the patient and was not searched for by the physicians. Choked disks, 
much diminished patellar reflexes, intense headache occurring periodi- 
cally, and tinnitus in the left ear were rather suggestive of the cere- 
bellum as the seat of the tumor, and Mingazzini says that if lateral 
homonymous hemianopsia had existed one might still think of cere- 
bellar tumor, as this phenomenon as a distant symptom has been 
observed in tumor of the cerebellum. 

Lumbar puncture was made in this case and the patient went into 
collapse the same evening and died the next morning. This procedure 
is dangerous in cases of tumor of the occipital lobe, cerebellum or 
medulla oblongata, and probably also when the tumor is situated else- 
where in the brain. 

A similar case has been observed by Mann. A tumor of the occipital 
lobe was found. Hemiataxia and the Babinski sign occurred on the 
same side as the tumor, and there was much in this case to suggest a 
tumor of the cerebellum. 

Oppenheim speaks of a case of Schultze in which a cerebellar tumor 
by pressure on the occipital lobe caused symptoms of a lesion in the 
occipital lobe. He refers to a case of his own in which a tumor of the 
precuneus caused cerebellar symptoms by pressure. A tumor of the 
cerebellum was observed by him to compress the descending horn of 
the lateral ventricle, and thereby to cause distention of this horn and 
interference with the function of the occipital lobe. I have seen dis- 
tention of one posterior horn of the lateral ventricle caused by a tumor 
within the pons. 

TUMORS OF THE PARIETAL LOBE. 

Tumors in this area of the brain may cause convulsions in the limbs 
and face of the opposite side by irritation of the adjoining motor area, 
weakness of the limbs of the opposite side from pressure upon the 



396 THE EYE AND KEKVOUS SYSTEM 

cortical motor centres, loss of stereognostic perception and of the sense 
of position, and loss or impairment of tactile sensation with less implica- 
tion of pain sensation, and awkwardness of movements in the limbs of 
the other side. The astereognosis and loss of the sense of position may 
be so great that the patient, as in a case under my care, may be unable 
to recognize any object placed in his hand of the opposite side when the 
eyes are closed, or have any knowledge of the position of the limbs of 
the opposite side, and may take the hand of the examiner believing it to 
be his own. In the case referred to the meningitis in plaques was found 
over and almost confined to the opposite parietal lobe. Ataxia of the 
limbs on the side opposite the lesion is to be expected if the sense of 
position is so impaired that the patient has no knowledge of the posi- 
tion of his limbs. I have, in several cases of lesion of the parietal lobe, 
observed the patient place the normal hand on his shoulder or some 
part of the opposite upper limb in searching for the hand of this side, 
and by following the limb down reach his hand. 

Although I believe that a lesion of the parietal lobe may cause ataxia, 
loss of stereognostic perception, and loss of the sense of position and 
some disturbance of tactile and possibly of pain sensation in the limbs 
of the opposite side, I have not been convinced that a lesion of the 
motor- area, or at least of the post-central convolution, will give none of 
these symptoms. The tendency now is to regard the post-central con- 
volution as having no motor function. 

Marinesco remarks that Jonesco removed a part of the cortical centre 
for the left upper limb in a man. After the operation the patient could 
move his shoulder and elbow very well, but not his hand. Nine months 
later the movements of the left hand were very imperfect, and tactile 
sensation, the sense of position, and stereognostic perception were 
diminished in the left hand. In another case Jonesco removed the 
centres of the face and upper and lower limbs of the right cerebral 
cortex. A year after the operation there was much weakness of the left 
side of the body, and the sense of position, stereognostic perception, and 
tactile sensation were affected in the left upper limb. Thermal and 
painful sensations were preserved. These operations were done for epi- 
lepsy, and in one case, at least, and probably in both, the cortical motor 
areas were determined by the electric current. 

I have observed loss of stereognostic perception caused by lesions 
much below the cortex of the brain, — viz., in a case of tumor of the pons 
(Potts and Spiller) and in a case of tumor compressing the medulla 
oblongata (case of Dercum) ; but where the symptoms indicate a tumor 
of the cerebrum that probably is cortical, I believe the loss of stereog- 



TUMORS AND OTHER BRAIN LESIONS 397 

nostic perception and of the sense of position and ataxia of the upper 
limb of the side opposite to that in which is the lesion indicate much 
implication of the parietal lobe. 

In 1898, at a meeting of the Philadelphia Neurological Society, 
I 9 referred to a case that I had seen in the service of Dr. Lloyd, at the 
Philadelphia Hospital, which seemed to show that the cortical represen- 
tation of the sense of position is in the parietal lobe. A man complained, 
during the night preceding his attack, of fatigue, headache, and inability 
to sleep. The following morning he fell to the floor on attempting to 
rise from his bed, but was not unconscious. When he was found he had 
paresis of the left limbs and of the left side of the face and tongue. 
Later the patient could raise his left upper limb above his head, but 
every movement of this limb was ataxic in an extreme degree, though 
the limb was not paralyzed. The mental condition of the patient pre- 
vented an examination of the condition of sensation. At the necropsy a 
hemorrhage was found in the right parietal lobe. The hemorrhagic 
area was about 2 centimetres in diameter, and extended inward in the 
form of a cone to the lateral ventricle, having its base in the cortex. It 
was situated about 4 centimetres from the longitudinal fissure and 
about 2 or 3 centimetres behind the Rolandic fissure. The brain 
had been cut into frontal sections about 1 centimetre apart, when 
I had an opportunity to examine it, and, although the injury to the 
cerebral tissue prevented an exact determination of the location of 
the hemorrhage, it was evidently in or very close to the supra- 
marginal gyrus. The ataxia was probably caused by a loss of the sense 
of position. 

This was probably one of the first cases reported in this country, 
showing that ataxia from a loss of the sense of position may be caused 
by a lesion of the parietal lobe. The case of Starr and McCosh 
preceded it. 

A case that Dr. C. K. Mills and I saw together, June 3, 1901, in 
consultation, the case being under the charge of Dr. Ida Richardson, 
affords evidence also of the localization of the stereognostic perception 
and of the sense of position in the parietal lobe. The patient's left upper 
limb was decidedly ataxic, paretic, and astereognostic, and the head was 
retracted and rigid. The necropsy was made by Dr. W. F. Hendrick- 
son. An area of depression, indefinite in outline, but about 6 centi- 
metres in diameter, was found in the parietal lobe of the right cerebral 
hemisphere just posterior to the fissure of Rolando. Palpation revealed 
less resistance at this point than over the surrounding tissue. On section 

9 Journal of Nervous and Mental Disease, January, 1S99, p. 43. 



398 THE EYE AND NERVOUS SYSTEM 

this area was found to be the seat of extensive softening. The process, 
implicated practically the entire right parietal lobe. 

The case reported by Dr. C. K. Mills/ which was seen by me and 
others in consultation, was one of the first to establish the localization 
of the stereognostic perception in the parietal lobe. A diagnosis of 
tumor of this lobe was made because of the loss of the sense of position, 
impaired cutaneous sensibility, astereognosis, and ataxia. The case 
has been so fully reported by Dr. Mills and is so well known that I need 
not refer to it further. 

Oppenheim, 11 after giving a description of his case of parietal tumor 
with operation, remarks that lesions of the parietal lobe cause ataxia 
of one or both limbs of one side. Irritative or paralytic motor symptoms 
are absent, or when they occur they are the result of pressure. The 
sensory disturbance is always a partial hemianesthesia, in that pain and 
temperature sensations are preserved, while tactile sensation and espe- 
cially the sense of position and the stereognostic perception are impaired 
or lost. Such a symptom-complex occurs when the lesion does not 
extend far into the white matter of the parietal lobe. He says, also, that 
the cases in which tumor of the parietal lobe has been so accurately diag- 
nosed that a radical operation was possible are very few. 

This case of Oppenheim was reported by him February 12, 1900, 
and published in the third number of the Mitteilungen, 

In his monograph 12 on tumors of the brain he says that in the ma- 
jority of cases of tumor of the parietal lobe sensory, irritative and 
paralytic phenomena were observed. He refers to Mills's case, but adds 
that the cases are not sufficient to permit us to regard the above-men- 
tioned phenomena as positive evidence of lesions of the j)arietal lobe. 
The evidences of the localization value of these symptoms are, however, 
rapidly accumulating. 

In March, 1901, Dr. C. W. Burr 13 reported a case in which there 
were slight hemiplegia, slight tactile anaesthesia, astereognosis, mind- 
blindness, loss of the temperature sense on one side, sensory aphasia 
varying in intensity, and mental dullness. In this case a tumor was 
found in the parietal lobe, pressing against but not invading the ascend- 
ing parietal convolution, partially destroying the angular gyrus, and 
involving almost the entire posterior parietal lobule. 

10 Mills, Keen, and Spiller, Journal of Nervous and Mental Disease, May, 1900, 
p. 244. 

11 Mitteilungen aus den Grenzgebieten der Medizin und Chirurgie, 1900, vol. vi. 
pp. 382, 383. 

ri Oppenheim, Die Geschwiilste des Gehirns, second edition, p. 116. 
13 The Amer. Journ. of the Med. Sci.. March, 1901, p. 306. 






Figs. 2, 3.— Extreme muscular atrophy, hemiplegia, and bilateral contractures from internal hydroce- 
phalus confined to one side of brain. 



TUMORS AND OTHER BRAIN LESIONS 399 

A tumor of the parietal lobe by growing downward may involve the 
internal capsule and the optic radiations, and thereby cause hemiplegia, 
hemianesthesia, and hemianopsia. 

TUMORS OF THE TEMPORAL LOBE. 

When the tumor is situated in the left temporal lobe, sensory aphasia 
from implication of the posterior part of the first temporal convolution 
may be expected. The' patient becomes word-deaf and is unable to 
speak connectedly; he may also be word-blind. The latter symptom, 
according to some neurologists, is to be explained by implication of the 
left angular gyrus. The right temporal lobe is a much more " silent 
area" than the left, at least in right-handed people, and a tumor here 
may grow to a considerable size before causing localizing symptoms. A 
tumor in either the right or left temporal lobe may by its growth cause 
pressure upon, or direct involvement of, the posterior limb of the inner 
capsule, with hemiplegia of the opposite side of the body. 

TUMORS OF THE MOTOR AREA. 

A growth in this region is more easily diagnosticated than one in any 
other part of the brain. Jacksonian motor epilepsy with paresis or 
paralysis of the face and limbs, and with motor aphasia, if the tumor is 
on the left side in a right-handed person, forms a symptom-group very 
characteristic of tumor in the central gyri. I have observed a case in 
which a gradually developing hemiplegia, with paresis of the other 
lower limb, resulted from internal hydrocephalus confined to one lateral 
ventricle. Such a case might be difficult to diagnose from one of sub- 
cortical tumor of the motor region (Figs. 2, 3). 

TUMORS OF THE FRONTAL LOBE. 

The frontal lobe may be seriously involved by a tumor without 
causing localizing symptoms, but it is probable that a growth is more 
likely to cause derangement of the higher intellectual functions when it 
is situated in this portion of the brain, especially in the left cerebral 
hemisphere. Judgment, power of concentration of thought, ability to 
understand what is read, etc., may show early impairment in cases of 
frontal tumor. 

Jacksonian convulsions in the limbs of the opposite side are not 
uncommon, because of the proximity of the motor area, and hemiplegia 
or monoplegia of the opposite side may also be produced by pressure 
upon, or direct implication of, the motor region of the brain. According 



400 THE EYE AND NERVOUS SYSTEM 

to the investigations of Griinbaum and Sherrington, the centres for the 
Kmbs and face are entirely in front of the central fissure. 

TUMORS OF THE OPTIC THALAMUS. 

According to some neurologists, we may expect as signs of a lesion 
of the optic thalamus a hemiplegia which in some cases may be slight, in 
others intense, but even then is likely to diminish in intensity, and often 
is less pronounced than is the hemianesthesia on the same side as the 
paralysis. These symptoms are explained by the theory that all sensory 
fibres pass through the optic thalamus, and therefore a tumor growing in 
this ganglion directly implicates the sensory tract, and causes a dis- 
turbance of motion, at first by pressure upon the internal capsule, and 
later by direct implication of this system of fibres. The case that I had 
the opportunity of studying with Dr. Dercum some years ago, in which 
objective disturbance of sensation had existed many years, and a cyst 
was found in the carrefour sensitif of Charcot and in the lenticular 
nucleus, without any implication of the optic thalamus, has led me to 
believe that the sensory fibres probably do not all terminate in the optic 
thalamus. In addition to the disturbances of motion and sensation 
mentioned, subjective disturbances of sensation, — viz., pain, also chorei- 
form or athetoid movements, forced laughter, hemianopsia, disturb- 
ances in the emotional innervation of the opposite side of the face in 
laughing and crying, and forced movements, — may be expected. 

In a case of abscess of the right optic thalamus, C. E. Beevor 14 ob- 
served severe pain in the occipital region of the same side, vomiting, 
double optic neuritis, with some weakness of the face and limbs of the 
opposite side, a tendency of the patient to rotate to the side of the 
lesion, in the direction of screwing-in, pleurosthotonus and a tendency 
to fall backward and to the right. 

He regards as symptoms of tumor of the optic thalamus occipital 
pain on the same side as the tumor, impaired mentality, weakness and 
nystagmus of ocular movements to the same side, weakness of the 
opposite limbs, rigidity of the limbs of the same side, staggering back- 
ward and to the same side, and attacks of rotation of the trunk with the 
face towards the same side. 

The involuntary movements found in cases of lesion of the thalamus 
may be ataxic, choreic, or of the nature of intention tremor. 

Charcot believed that hemichorea is produced by a lesion of certain 
fibres of the corona radiata, or of a portion of the posterior limb of the 
inner capsule in front of the carrefour sensitif. Gowers attributed the 

"Beevor, Brain, 1898, vol. xxi. 



TUMORS AKD OTHER BRAIX LESIOXS 401 

heniichorea to a lesion of the optic thalamus ; Kahler and Pick to irri- 
tation of the pyramidal tract, but Bonhoeffer 15 believes that hemichorea 
depends upon a lesion of the superior cerebellar peduncle, and he at- 
tempts to show that in most of the cases with necropsy in which hemi- 
chorea was present these peduncles were implicated. 

The diagnosis of tumor of the optic thalamus not infrequently is 
one of great difficulty and in some cases it is impossible. The symptoms 
may be those of a cerebellar tumor as in a case observed by Douglas 
Stanley, 16 in which a large glio-sarcoma was found occupying the whole 
of the left optic thalamus, causing compression of the internal capsule 
and displacement outward of the lenticular nucleus, and projecting 
into the lateral ventricle, so as to cause slight pressure on the opposite 
optic thalamus. The patient was a little weak in the right arm and 
leg, and had difficulty in controlling volitional movements of these limbs. 
He had a coarse, irregular tremor of the right arm. His gait was un- 
steady and he reeled to the right side. On attempting to stand with his 
feet together he was liable to fall to the right side. Occasionally con- 
vergent strabismus appeared on the right side, and occasionally he had 
internal squint with the left eye varying in degree, so that there was 
alternating converging strabismus, and it was often difficult to say which 
external rectus was most affected. This case shows that paralysis of an 
external rectus may be a distant symptom, and I have seen it in a case 
of tumor of the parietal lobe. 

The occurrence of Jacksonian epilepsy from a tumor situated deeply 
in the brain may cause a mistake in diagnosis, but in rare instances con- 
vulsions of this character may occur, as in a case reported by J. M. 
Clarke, in which a tumor was confined to the optic thalamus. * Paralysis 
nowhere was absolute, and in the face was slightly marked, and there 
was no deviation of the tongue. There was rigidity but no ankle clonus, 
nor exaggeration of reflexes. Loss of control over bladder and rectum 
existed. In these respects the group of symptoms, he says, corresponded 
to those observed by Bastian. In addition there was left ptosis, dilated 
pupils, with loss of reaction to light, turning of the head first to the left 
and afterward to the right side, with mobile spasm of the right arm and 
leg, and vasomotor phenomena of the right arm. 

In Clarke's case the head looked towards the side of the lesion in the 
earlier stages and only subsequently was directed towards the healthy 
side. 

According to the experiments of SchifT and Xothnagel in unilateral 

15 Bonhoeff er, Monatsschrift fiir Psychiatrie und Neurologie, vol. i. p. 6. 
"Stanley. Brain, 1898. vol. xxi. 
26 



402 THE EYE AND NEKVOUS SYSTEM 

lesions of the thalamus the head looks towards the healthy thalamus, 
while on the side of the healthy thalamus the flexors of the upper 
extremity, and on the side of the lesion the extensors of the upper 
extremity appear contracted. 

In Clarke's case the positions of the right arm and leg exactly corre- 
sponded to those commonly observed in ordinary cases of hemiplegia 
from hemorrhage into the internal capsule, and he believes they were 
accounted for by pressure of the tumor on the inner capsule. During 
the short time the patient lived after an operation the symptoms of 
paralysis and spasm disappeared in the face and arm, and almost 
entirely in the leg. Any effect obtained by operation was believed to be 
by relief of pressure, and would seem to indicate that pressure on the 
internal capsule was the essential agent in the production of paralysis 
and spasm. 

Clarke 17 describes in this case convulsive seizures, beginning in the 
right arm and affecting the right arm and leg, lasting for two or three 
minutes and followed by rigidity of these limbs for the same length of 
time, or of spasm confined to the right arm occurring at irregular in- 
tervals. These attacks seem to have been of the Jacksonian type, 
although the lesion was in the optic thalamus. Later the spasmodic 
movements of the right upper limb were continuous. He describes also 
an intention tremor among the early symptoms. 

Usually Jacksonian motor epilepsy indicates a lesion in or near the 
motor cortex, but I have found this sign in a case of my own, caused by 
a tumor deep within the white matter of the brain but above the internal 
capsule. 

A tumor of the optic thalamus does not always cause disturbances 
of vision, although it would be expected to do so because of the probable 
situation of visual fibres within the pulvinar, and because of the 
liability to interference with the flow of cerebrospinal fluid through 
the aqueduct of Sylvius. In a case observed by Byrom Bramwell, 18 
a diagnosis of cortical gumma in the position of the right lower limb 
centre was made on account of tenderness over the leg and arm centres 
on the right side; and of attacks of Jacksonian epilepsy occurring 
several times a day, the spasm beginning in the left big toe; and 
because of exaggeration of the left knee-jerk. Other symptoms of 
tumor were present. An examination at the necropsy revealed a glio- 
sarcoma about the size of a small egg in the right optic thalamus and 
encroaching slightly upon the motor fibres of the pyramidal tract. The 

17 J. M. Clarke, Lancet, 1891, p. 1283. 

18 Byrom Bramwell, Brain, 1899, vol. xxii. p. 49. 



TUMORS AND OTHEE BRAIN LESIONS 403 

tumor was thought to have produced irritation of the leg fibres of the 
internal capsule, but it seems very strange that a tumor so deep within 
the brain as in this and in Clarke's case should have caused Jacksonian 
epilepsy. Bramwell says that in four cases in which the optic thalamus 
was invaded by a new growth he was unable to detect any definite symp- 
toms due to a lesion of this area, unless paroxysms of flushing, which 
Avere very marked in one case, were due to this cause. 

In a case of tumor of the posterior portion of the left optic thalamus 
and pressing on the inner capsule, reported by Major, 19 there was no 
optic neuritis and no color-blindness. The symptoms were paralysis 
of the right limbs and right side of the face, numbness of the right 
side, dilatation of the right pupil and right homonymous hemianopia. 

TUMORS OF THE CORPORA QUADRIGEMINA 
AND CEREBRAL PEDUNCLES. 

If we recall the structure of the region of the corpora quadrigemina 
we can understand that ocular palsies are usually among the early mani- 
festations of tumor of this part of the brain. The nuclei of the oculo- 
motor and trochlear nerves lie near the aqueduct of Sylvius, and there- 




Fig. 4.— Fibroma on the corpora quadrigemina. The right cerebral hemisphere, and the posterior 
part of the pons have been removed. 

fore pressure upon these nuclei, or direct implication of them by a 
tumor causes partial paralysis of the muscles of the eyeball on each side. 
Ataxia may be attributed to the involvement of the superior cerebellar 



Brit. Med. Joura., April 16, 1892, p. 818. 



404 THE EYE AKD NERVOUS SYSTEM 

peduncles which decussate below the aqueduct and form the red nuclei. 
Disturbance of hearing may also be expected, and may be explained by 
the implication of the posterior colliculi of the corpora quadrigemina. 
The inner muscles of the eye, the iris and ciliary muscles, may escape, 
because the position of the nuclei for these muscles is probably in ad- 
vance of those for the external muscles of the eyeball, in fact, rigidity 
of the iris is not a common sign of tumor in this part of the brain, and 
ptosis from involvement of the levator palpebral also may be absent. 
Inequality of the pupils has been observed from tumor of the corpora 
quadrigemina. Occasionally absence of convergence-reaction and paral- 
ysis of accommodation have been seen. Oppenheim reports recurrent 
ophthalmoplegia. 

In Ivolisch's case of tubercle of the corpora quadrigemina and pons 
both oculomotor nerves were paretic, but the pupillary reaction was 
preserved. The tumor did not extend forward beyond the corpora 
quadrigemina, and probably for this reason the pupillary reaction was 
preserved. 

In a case of tuberculous tumor confined to the corpora quadrigemina 
reported by Bristowe, the tumor lay between the upper surface of these 
bodies and the aqueduct of Sylvius. All the movements of the eyes were 
more or less imperfect, except possibly downward movement. The 
irides reacted well to light, but reaction to accommodation could not be 
determined. Mr. Nettleship, who made the ophthalmological examina- 
tion, thought that the centres for the iris and ciliary muscles had 
escaped. This case also seems to show that these centres are in advance 
of the other oculomotor centres. 

In B. Sachs's case of tubercle of the corpora quadrigemina there 
was almost complete bilateral external ophthalmoplegia, but during the 
entire period of observation the ciliary muscles and irides remained 
exempt. Sachs regards this as striking proof that the nuclei of these 
muscles are at some distance from those governing the external ocular 
muscles. 

The internal ophthalmoplegia in Gordinier's case from a lesion far 
back in the oculomotor nuclei is remarkable, as the nuclei for the in- 
ternal ocular muscles are usually placed well forward, by L. Bruns, in 
the median side of the optic thalami. 

In a case of tumor which seems to have been confined to the aque- 
duct of Sylvius, and was reported by J. Collins, 20 the gait was like that 
of an intoxicated person, but there was no ocular palsy and hearing was 

20 Collins, Amer. Jour, of the Med. Sci., 1895, vol. ex. p. 420. 



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TUMORS AND OTHEK BRAIN LESIONS 405 

good. We may assume that pressure from the tumor was not sufficient 
to cause many symptoms. The third and lateral ventricles were much 
distended, but the fourth ventricle contained no fluid and presumably 
was not dilated. The case resembles one to which I have referred in my 
section on tumors of the occipital lobe, of occlusion of the aqueduct of 
Sylvius by proliferation of the neuroglia. 

Associated Ocular Palsies. 21 — Disturbances in the associated 
movements of the eyes (Blicklahmung) afford a sign of localizing value 
in lesions of the brain, and yet this subject has received comparatively 
little attention. Von Kornilow, who has written the best recent paper 
on these palsies, remarks that clinical cases carefully studied are rare, 
and even more so are those with necropsy. (See Chapter V, p. 266.) 

The opinion of Parinaud and Sauvineau that upward or downward 
associated ocular paralysis is always associated with paralysis of con- 
vergence is incorrect, as shown by a case reported by Parinaud himself, 
by a case reported by von Kornilow, and by other cases. It is true, how- 
ever, as Parinaud pointed out in 1883, that in paralysis of associated 
ocular movements diplopia often is absent. Indeed when in such palsy 
diplopia later appears it may be a sign of improvement, as it was in 
a case observed by me, where the disappearance of the palsy in one 
eye caused diplopia, soon followed by complete recovery. Although 
paralysis of associated ocular movement of any form is usually a sign 
of grave significance, one should be careful about giving a fatal prog- 
nosis. In a case reported by Raymond and Cestan 22 the paralysis 
lasted ten years, and in a number of cases complete recovery has oc- 
curred, while in others improvement has been very marked, as in some 
of the cases studied by me. 

The evidence is strong that paralysis of associated lateral movements 
of the eyeballs is indicative of a lesion of the posterior longitudinal 
bundle near the sixth nucleus. The external rectus muscle on the side 
of the lesion may be more affected than the internal rectus of the other 
eye, and this according to Parinaud 23 is the rule. He mentions also 
that the movement of convergence may be greater in the eye in which 
the external rectus is paralyzed, because of the paralysis of the external 
rectus. 

The disturbances of the upward or downward associated movements 
have been studied less than those of lateral associated movement. The 

21 Adapted from the author's Presidential Address, read at the meeting of the 
American Neurological Association, June, 1905, and published in the Journal of 
Nervous and Mental Disease. July and August, 1905. 

"Revue Neurologique, 1901, p. 70. 

23 Archives de Neurologie, vol. v. No. 14, 1S83, p. 145. 



406 THE EYE AND NERVOUS SYSTEM 

former, though usually acquired, may be congenital, and Oppenheim 24 
has observed congenital paralysis of associated upward movement. 
Uhthoff 25 speaks of paresis of upward associated movement with slight 
impairment of movement in other directions, occurring in multiple 
sclerosis. 

We have reason to believe that the cerebral cortex exerts some con- 
trol over the associated movements of the eyeballs ; especially strong 
is the evidence concerning lateral conjugate movements. Conjugate 
deviation of the eyes has been studied within the past few years by 
Klaas, 26 and from his investigations of the literature, it appears that 
this phenomenon was first observed by Andral (1834). It was studied 
by Duplay, Durand-Fardel, and others, but Prevost showed that con- 
jugate deviation of the eyes often occurs in hemiplegia and has nothing 
to do with strabismus, as Andral believed; he showed further that it 
is often associated with deviation of the head; that the deviation is 
toward the lesion when the lesion is cerebral, and away from the lesion 
when it is in the isthmus. Landouzy believed that irritation and 
paralysis produce deviation in opposite directions when the lesion is 
in the cerebrum, and Grasset showed that the patient looks at his 
paralyzed limb when the lesion is a paralyzing one in the brain stem, 
and at the lesion when it is an irritating one, and in this part of the 
brain. Wernicke assumed that the rapid disappearance of conjugate 
deviation of the head and eyes is owing to the partial control of the 
lateral ocular movements by the cerebral hemisphere of the same side, 
and to the action of this hemisphere in place of the injured opposite 
cerebral hemisphere. Wernicke believed that conjugate deviation of 
the eyes is caused by a lesion of the lower parietal lobe, and he reported 
three cases of lesion in the lower part of each parietal lobe in which 
voluntary lateral associated ocular movements were lost, pseudo-ophthal- 
moplegia, although in another case with similar lesions disturbance 
of the ocular movements was not observed. 

The evidence concerning a centre for lateral movements of the 
eyes in the lower parietal region or angular gyrus is conflicting. There 
is more evidence that a centre for lateral associated ocular movements 
is situated in the posterior part of the frontal lobe. 

The recent investigations of Grunbaum and Sherrington by elec- 
trical irritation have again demonstrated that a centre for ocular 



^Lehrbuch der Nervenkrankheiten, 4th edition, vol. ii. p. 706. 
^Archiv. fur Psychiatrie, vol. xxi. 1890, p. 379. 

26 Uber konjugierte Augenablenkung bie Gehirnkrankheiten, Inaugural Dis- 
sertation, Marburg, 1898. 



TUMORS AND OTHEE BRAIN LESIONS 407 

movements probably exists in the frontal lobe. Pathological alteration 
of the frontal lobe has given evidence to the same effect. In a case 
of cerebral syphilis with severe lesion of the right frontal lobe, reported 
by Karl Schaffer, 27 paralysis of associated movement of the eyeballs 
to the left occurred, and Schaffer concludes from a study of this case 
that a centre for lateral associated movements exists in the posterior 
part of the second frontal convolution, although he does not deny that 
there may be another and similar centre in the supramarginal and 
angular gyri. 

Hemianopsia cannot be regarded as the cause of conjugate devia- 
tion in all cases, although much has been attributed to it recently by 
certain French writers. Dejerine and Roussy 28 have demonstrated 
that it is not necessarily the cause of the deviation, inasmuch as devia- 
tion existed in a patient of theirs who had been born blind and whose 
cortical visual zone had not been developed. 

So far the knowledge gained relates chiefly to associated lateral 
movements, but we know much less concerning a cortical centre for 
upward or downward associated movements. 

The views of Parinaud and some other writers on this subject lack 
confirmation. They believed that there is a centre for ocular move- 
ments in the occipital lobe and a more important one in the frontal 
lobe. The inferior part of the latter centre controls the upward move- 
ment of the eyes, the superior part controls the downward movement 
of the eyes, the intervening part controls the lateral movements. It is 
true that lateral associated movements have been impaired with paraly- 
sis of upward or downward associated movements in a number of 
instances, as they were in some of my cases, but a lesion in the vicinity 
of the corpora quadrigemina will better explain this form of paralysis 
than a lesion of the cerebral cortex. Teillais says, regarding his own 
case, it would be unreasonable to regard the lesion as cortical, because 
if the lower part of the cortical centre controls the upward move- 
ments, and the upper part the downward movements, then the whole 
of this centre except the middle portion must have been destroyed, 
as the lateral movements were preserved. Such a view cannot be 
accepted. 

Paralysis of lateral associated movement from cortical lesion, as 
seen in the conjugate deviation of the eyes, is always, so far as I know, 
transitory, and the persistence of such paralysis points to a lesion of 
the posterior longitudinal bundle. We have little anatomical or 

27 Neurologisches Centralblatt, Nov. 16, 1904, p. 1035. 

28 Revue Neurologique, Feb. 15, 1905, p. 161. 



408 THE EYE AND NEKVOUS SYSTEM 

pathological evidence that is really valuable concerning the existence 
of a cortical centre for upward or downward associated movements, but 
in reasoning from analogy we must assume that such a centre or centres 
exist. The clinical case that Parinaud 29 reported in 1892 as indi- 
cating a lesion of the frontal centre, is in the light of our present knowl- 
edge unsatisfactory because of the association of homonymous lateral 
hemianopsia. 

Parinaud reported the case before the Society of Ophthalmology, 
April, 5, 1892. The man had paralysis of the lower left part of the 
face, of the left half of the tongue, and temporarily of the left hand. 
He had almost complete paralysis of the elevators of the eyeballs, with 
a little reduction in the extent of the lateral movements. Convergence 
was well performed. (Here Parinaud presents a case of paralysis of 
upward associated movement with preservation of convergence.) 
Downward movement was normal. Parinaud believed there was a 
lesion in the right frontal lobe, at the level of the lower part of the 
Polandic fissure, involving the centres for the face, tongue, hand, and 
associated movements of the eyeballs. 

This patient had transitory partial left homonymous hemianopsia, 
and this was supposed to be the result of a lesion near the visual zone, 
which occupies, according to the teaching of Parinaud in 1892, all the 
occipital lobe, and extends to near the ascending parietal convolution. 
This opinion we now can hardly accept. 

Hysteria may cause paralysis of the associated ocular movements, 
but there is no pathognomonic sign of this form of the palsy, and in 
some instances a correct diagnosis may be extremely difficult or even 
impossible. No case presents this difficulty more clearly than the one 
reported by Crouzon, Marie, and Babinski 30 before the Neurological 
Society of Paris on three different occasions in 1900 and 1901. 

Crouzon presented the case as one of spasmodic tic, later Babinski 
and Parinaud expressed the opinion that it was organic and caused by 
a supranuclear lesion, and still later Marie presented the case with the 
diagnosis of a purely functional disorder, a neurosis, as a spasm of 
the elevators of the eyeballs. When the patient threw his head as far 
backward as possible and followed with his eyes a finger lowered slowly 
the eyeballs moved downward, but if his head were erect and he at- 
tempted on command to look at his feet, he bent his head forward and the 
eyeballs went forcibly upward. After thirty or forty seconds the spasm 
disappeared and the eyeballs returned to the normal position. Marie 

29 Annales d'Oculistique, vol. cvii. p. 283, 1892. 
30 Revue Neurologique, 1901, p. 428. 



TUMOKS AND OTHEE BKAIN" LESIONS 409 

concluded from this that there must be a spasm of the elevators and 
not a paralysis of the depressors. The disorder had occurred after an 
apoplectic attack lasting seventeen hours, and the man had become 
blind. During the attack he had not had stertorous breathing nor pas- 
sage of urine or faeces, and this Marie regarded as remarkable if there 
were an organic lesion. The coma disappeared suddenly, but the patient 
was delirious for several weeks and did not recognize anyone or his 
surroundings. This Marie regarded as like hysteria. The speech was 
slow and hesitating, and the visual fields were contracted. 

Parinaud pointed out that the downward movement in following 
a finger is a reflex act and better performed than a voluntary move- 
ment, and this he says he has observed in hysteria, but when the 
condition is hysterical there is no inconvenience from the ocular dis- 
turbance, just as there is no inconvenience from the contraction of the 
visual fields in hysteria, and Parinaud has never seen a hysterical 
patient incline the head forward in order to make use of the superior 
part of the champ du regard (Dr. de Schweinitz suggests as a suitable 
translation for this term, field of fixation). The dissociation between 
the voluntary and reflex movements is not always hysterical. Pari- 
naud, has pointed out that spasm may occur in sound ocular muscles 
as the result of paralysis of other ocular muscles when the patient is 
required to look in different directions. A paralysis confined to one 
eye may be the cause of spasm in the other, so that the sound muscles 
may appear to be affected. In these cases the paralysis is peripheral, 
but even in associated palsies it is not rare to see disturbance of the 
antagonistic muscles. In conjugate lateral paralysis from lesion of 
one sixth nucleus there is almost always nystagmus when the patient 
looks toward the sound side, and this nystagmus may be so pronounced 
that it may be difficult to say which side is affected. This I also have 
noticed in a case of complete paralysis of lateral associated movements 
on one side and of partial paralysis on the other. In the case which 
called forth so much discussion in the Neurological Society of Paris 
the visual fields were contracted only in the inferior portion and not 
concentrically, therefore the contraction was not like that of hysteria. 
The champ visual (visual field) Parinaud distinguishes from the champ 
du regard (field of fixation). He believes that if the cortical centre for 
associated movements of the eyes in the frontal lobe be destroyed, the 
voluntary associated movements will be lost, but the reflex associated 
movements will be preserved, and yet little, if any, clinical evidence 
in support of this opinion can be found. 

Babinski pointed out that in the case under discussion the down- 



410 THE EYE AND NERVOUS SYSTEM 

ward movement of the eyes was imperfect with the head thrown back. 
He quoted the case reported by Schroder 31 in which only paralysis 
of the associated downward movement persisted, and the eyes could 
not be lowered below the horizontal plane. Any attempt to look down- 
ward caused a spasm of the elevators and the eyes turned upward. 

Oppenheim 32 has observed cases of pseudobulbar paralysis in which 
lateral movements of the eyeballs were impaired in voluntary innerva- 
tion, but were preserved when the patient tried to follow an object or 
to turn toward the direction from which a sound came. 

All the pathological evidence that I have been able to obtain in 
cases of persistent palsy of associated upward or downward movement 
is indicative of a lesion near the aqueduct of Sylvius. It is extremely 
doubtful whether a lesion confined to the corpora quadrigemina and 
causing no pressure on the surrounding parts ever causes paralysis of 
associated ocular movements, and those who favor such a view have 
not produced proof of a supranuclear centre in this part. Bern- 
heimer 33 says Topolanski showed that electrical irritation of the cor- 
pora quadrigemina of rabbits does not produce ocular movements, and 
that these parts could be removed without producing symptoms. He 
believed that there is a centre for ocular movements near the oculo- 
motor nucleus. 

Bernheimer, from experiments on monkeys, has shown that the 
ocular movements do not depend on the integrity of the corpora quad- 
rigemina, that this part of the brain does not contain a reflex centre 
for these movements, and that after destruction of this part the ocular 
movements are normal. He obtained synergic ocular movements from 
irritation of the angular gyrus both before and after he had removed 
the corpora quadrigemina. He thus refutes Prus's statements that a 
centre for ocular movements exists in the corpora quadrigemina. 

Cases have been reported in which the corpora quadrigemina have 
been destroyed without disturbance in the movements of the eyeballs 
(Weinland, Seidel, Ruel, Nissen, cited by von Kornilow). 

In a case that I have had the opportunity of studying there was a 
complete paralysis of right associated lateral movements, and great 
impairment of left and of upward associated movements. The paraly- 
sis of lateral associated movements is to be explained by the involve- 
ment of both posterior longitudinal bundles. If we assume that the 



81 Quoted by Teillais, Bui. de la Soc. Fran, d' Ophthal., 1899, p. 415. 

32 Neurologisches Centralblatt, 1895, p. 40. 

33 Wiener klin. Wochenschrift, No. 52, 1899, p. 1310. 



TUMORS AND OTHER BRAIN LESIONS 411 

nuclei of the superior rectus and inferior oblique muscles are in the 
posterior part of the oculomotor nucleus, we can understand why 
marked impairment of upward movement was present in this case, but 
this is contrary to the views held by some concerning the position of 
these groups of nerve cells. 

Siemerling 34 has recently remarked that the numerous experi- 
mental studies of Bach, Bernheimer, van Gehuchten, and van Biervliet 
have not led to uniform conclusions. The oculomotor nucleus is merely 
the place for the transference of impulses and we are not in a position 
to state which parts are concerned with the innervation of the individual 
muscles. 

Another of my cases is further evidence that the nuclei of the 
superior rectus and inferior oblique muscles may be in the posterior 
part of the oculomotor nucleus, because there was paresis of upward 
associated movement and the nerve-cells of the oculomotor nuclei were 
not diseased, but the posterior part of the nuclei was affected by press- 
ure from the tumor in the pons, and the aqueduct of Sylvius was com- 
pressed upon the left side. The tumor did not invade the nuclei of 
the third and fourth nerves. The paralysis of left associated lateral 
movement is explained by the almost complete destruction of the left 
half of the pons by a tubercle, and thereby involvement of the posterior 
longitudinal bundle. The left sixth nerve was also much degenerated. In 
this and a similar case studied by me, downward movement was normal. 
This would seem to be evidence that the nucleus of the inferior rectus 
muscle must be in advance of those for the superior rectus and inferior 
oblique muscles, as in each case the tumor grew from the pons and did 
not invade the oculomotor nucleus. 

If this is the correct position of these nuclei, a lesion developing 
ventrally in the pons as shown in two of my cases may by pressure 
upon the posterior part of the oculomotor nuclei cause paralysis of 
associated upward movement, as a result of injury of the nuclei for 
the superior rectus and inferior oblique muscles, and it would not be 
at all necessary to assume the existence of a co-ordinating centre for 
upward associated movement. Paralysis of downward associated move- 
ment depends on impairment of the inferior rectus and superior ob- 
lique muscles, these having cells of origin in two distinct nuclei. There 
must be a close association between the nucleus of the superior oblique 
and that of the inferior rectus muscle of the same side, and possibly 
of the opposite side. It is probable therefore that a lesion of the nuclei 
of the inferior rectus muscles and of the fibres connecting them with 

3 *Archiv. fur Psychiatrie, vol. xl. No. 1, p. 61. 



412 THE EYE AKD NERVOUS SYSTEM 

the nuclei of the trochlear nerves would cause paralysis of associated 
downward movement, and it seems unnecessary to assume that a co-ordi- 
nating supranuclear centre for upward or downward movements exists 
in the corpora quadrigemina or their vicinity. Indeed, two of my 
cases show that such a centre is improbable, because in each case the 
lesion causing paresis of upward associated movement was posterior 
to the oculomotor nucleus, and did not implicate the corpora quadri- 
gemina. Three of the cases of the series studied by me afford further 
evidence against a centre for co-ordinate ocular movements in the cor- 
pora quadrigemina. In one the paralysis at first was only in upward 
movements, later also in lateral associated and in downward associated 
ocular movements. In this case, therefore, a paralysis at first confined 
to one form of associated movement later extended to other forms. In 
another case the paralysis of upward associated ocular movements dis- 
appeared, leaving only paresis of the left superior rectus, and finally 
this also disappeared. In another case the associated palsy dis- 
appeared, leaving paralysis of muscles only on one side. It is very 
common to have a paisy of one or more branches of the oculomotor 
nerve in association with paralysis of associated upward or downward 
movement, and this is more easily explained by a lesion of the oculo- 
motor nucleus than by a cortical lesion. Cortical centres control asso- 
ciated movements, not isolated muscles. 

The paralysis of the sixth nerve occurring with paralysis of asso- 
ciated upward or downward movements has been attributed by Licht- 
heim to pressure upon the nerve at the base of the brain. The opinion 
held by Parinaud, although supported by Duval, Laborde, and Graux, 
can hardly be accepted, — viz., that the sixth nucleus gives nerve fibres 
to the internal rectus muscle, and that this muscle has a double inner- 
vation, the sixth nerve for lateral movement, the third for convergence. 

The assumption of a basal co-ordinating centre above the oculomotor 
nucleus for ocular movements seems forced. The lateral associated 
ocular movements can be well explained by the connection formed by 
the posterior longitudinal bundle between the nucleus of the branch 
to the internal rectus muscle and the nucleus of the sixth nerve. It 
is reasonable to suppose that certain fibres connecting the different parts 
of the oculomotor nucleus with one another, or connecting the group 
of nerve cells innervating the inferior rectus with the nucleus of the 
trochlear nerve has a co-ordinating function similar to that of the pos- 
terior longitudinal bundle, and that this function may be disturbed 
by a small lesion. It is striking that the paralysis of downward asso- 
ciated movement (necessitating the implication of two separate nuclei 



TUMORS AND OTHEE BRAIN LESIONS 413 

of ocular muscles) without paralysis of upward associated movement 
is exceedingly rare, far more so than is the isolated paralysis of upward 
associated movement. 

Conjugate deviation of the eyeballs from lesions of the pons is not 
common when the lesion is confined to one side of the pons. In a case 
of hemorrhage into the tegmentum of the pons that I have had the 
opportunity to study and the brain from which I have examined, in- 
ward deviation of only the right eye occurred from paralysis of the 
sixth nerve. One gets the impression that conjugate deviation of the 
eyeballs away from the side of the lesion is common in lesions of one 
side of the pons, but in four cases of lesions of this part observed by 
me (three of tumor and one of hemorrhage) causing paralysis of the 
external rectus muscle of the same side, I have looked for the conjugate 
deviation, but failed to find it. It is probable that in most cases the 
side of the pons opposite to the lesion is directly or indirectly involved. 

Yon Monakow 35 believes that when the lesion implicates the sixth 
nucleus of one side, apart from the paralysis no disturbance, — i.e., 
no deviation occurs, unless it is the result of secondary contracture. 
I believe that conjugate deviation is rare even as a late sign of paraly- 
sis of one sixth nucleus. Of course any involvement of both sixth 
nuclei would make conjugate deviation impossible, and in one of my 
cases both these nuclei were affected. 

In a case of softening of the dorsal part of the left side of the pons 
reported by Albert Ransohoff 36 there was complete paralysis of the 
associated ocular movement to the left and of convergence movement 
of the right eye. The right external rectus muscle was not overactive, 
but the left internal muscle was in marked spasm. The left abducens 
nucleus was involved in the lesion, the right escaped, but the right 
posterior longitudinal bundle was involved. Ransohoff mentions that 
Wernicke and Jolly have shown that paralysis of the external rectus 
muscle does not in itself cause contraction of its antagonist, the internal 
rectus, and that in the cases of these authors, as well as in his own, there 
was a lesion of the opposite posterior longitudinal bundle. In Wer- 
nicke's case a tumor had destroyed the left adducens nucleus and invaded 
the right side of the pons. Both eyeballs were persistently turned to 
the right, especially the left eyeball. 

According to the study of the literature by Ransohoff, contracture 
of the unparalyzed antagonistic muscles, but not always in both eyes, 
and more in the internal rectus muscle on the side of the lesion than 



Gehirnpathologique, p. 335. 

Archiv. fur Psychiatrie, vol. xxxv. 1901, p. 416. 



414 THE EYE AND NERVOUS SYSTEM 

in the external rectus muscle on the side opposite to the lesion, was 
observed in the cases of Wernicke, Jolly, Graux, Meyer (the lesion 
did not extend to the mid-line), Spitzer (slight, and after one-half 
year duration of the lesion), Mierzejewsky and Rosenbach, Gebhard, 
and Ransohoff. 

RansohofT says that if the posterior longitudinal bundle, as is as- 
sumed by most authors, contains a part of the voluntary fibres for the 
internal rectus muscle of the opposite side, a lesion of these fibres, as 
of the pyramidal fibres, must cause spastic contracture of the muscle 
they innervate. Therefore, according to RansohofT, contracture of the 
internal rectus muscle on the side of the lesion indicates that the pos- 
terior longitudinal bundle of the opposite side is also involved, but 
this contracture probably could not exist if the nucleus of each abdu- 
cens were destroyed. 

In four of my cases both posterior longitudinal bundles must have 
been much affected and conjugate deviation did not occur in any. 

In one case in which paralysis of lateral movements was present, 
the movement of either eyeball to the right or left was distinctly greater 
when one eye was covered than in associated lateral movements. The 
individual movements of the eyeballs were therefore greater than the 
associated movements. 

One of my cases was especially interesting in that syphilitic infec- 
tion was positive. The paralysis of associated movement was peculiar ;. 
the downward movement was completely lost in the right eye and much 
impaired in the left; the upward movement was slight in the right 
eye and completely lost in the left; the lateral associated movements 
were nearly normal. Almost all the ocular movements were regained 
except the downward m'ovement of the right eye. It is remarkable 
that the complete paralysis in the associated movements was in the- 
downward direction in one eye and in the upward direction in the 
other, although the associated movement was very feeble. It is re- 
markable that the associated palsy disappeared leaving paralysis of 
downward movement of the right eye. 

In four cases of my series with necropsy the pupil was smaller on 
the same side as the lesion of the pons. This alteration of the size* 
of the pupil from a lesion of the pons is difficult to explain, but the 
presence of the sign in four cases shows that it is of diagnostic signifi- 
cance, and also that the fibres controlling the iris pass through the pons. 

In at least two cases nystagmus was present in looking away from 
the side of the chief lesion. This was caused by weakness of the oppo- 
site external rectus muscle from pressure upon or direct involvement 



TUMOBS AND OTHEB BBAIN LESIONS 415 

of the nucleus of the abducent nerve. This sign may be expected when 
a lesion affects the dorsal part of the pons and destroys the function 
of one abducent nucleus and weakens that of the other. Nystagmus 
may surely be the result of paresis of ocular muscles. 

I have studied thirty-eight cases of paralysis of upward or down- 
ward associated movement reported in the literature, not including 
nine of my own. In many cases certain symptoms are not referred to, 
so that statements regarding the frequency of symptoms are incomplete. 
Von Kornilow used only twenty cases of the twenty-seven to which he 
found references in drawing his conclusions. As a result of my study 
of these forty-seven cases, — 

Paralysis of upward associated movement without paralysis or 
paresis of downward associated movement was found in twenty-six 
cases (Henoch, Priestley Smith (2), Nieden, Gowers, Parinaud (2), 
Ormerod, Keinhold, Thomsen, Lichtheim (3), Hoesslin, Bruner, Mills, 
Nogues and Sirol, Kornilow, Posey, Parkinson, Cases 1, 2, 4, 5, 6, 8 
of my series). 

Paralysis of upward associated movement without paralysis or pare- 
sis of downward associated movement was found in sixteen cases (Wer- 
nicke, Parinaud, Hope, Nothnagel, Eisenlohr, Verrey, Sharkey, Sau- 
vineau, Teillais, Gordinier, Baymond and Cestan (slight), Kornilow, 
Cases 3, 4, 7, 9 of my series). 

Paralysis of upward associated movement with impairment of 
lateral movement, often developing later, was found in fifteen cases 
(Henoch, Priestley Smith (2), Lichtheim (3), Parinaud, Poulard, Bay- 
mond and Cestan, Kornilow, Parkinson, Cases 1, 2, 3, and 4 of my 
series). 

Paralysis of upward associated movement without impairment of 
lateral movement was found in twenty- two cases (Wernicke, Nieden, 
Gowers, Parinaud (2), Ormerod, Hope, Thomsen, Sharkey, Sauvineau, 
Hoesslin, Bruner, Teillais, Gordinier, Nogues and Sirol, Kornilow, 
Posey, Cases 5, 6, 7, 8, and 9 of my series). 

Paralysis of downward associated movement without paralysis of 
upward associated movement was found in five cases (Gee, Poulard, 
Curzon-Marie and Babinski, Basevi, Schroder), but in none of my nine 
cases. 

The reaction of the iris was found to be impaired in fourteen cases 
(Henoch, Gowers, Parinaud, Thomsen, Eisenlohr, Lichtheim, Sharkey, 
Sauvineau, Hoesslin, Gordinier, Mills, Kornilow, Posey, Case 9 of my 
series), and is said to have been normal in four cases (Parinaud, Teil- 
lais, Poulard, Baymond and Cestan), but in many cases no statements 



416 THE EYE AKD NERVOUS SYSTEM 

regarding the iritic reflex are made, and in all my nine cases the iritic 
reflex was preserved. 

The optic nerve was found to be affected in fifteen cases (Gowers, 
Thomsen, Eothnagel, Gee, Eisenlohr, Lichtheim (3), Sharkey, Hoess- 
lin, Gordinier, Cases 1, 5, 7, and 9 of my series), and normal in twelve 
cases (Wernicke, Nieden, Parinaud (2), Ormerod, Reinhold, Sauvi- 
neau, Teillais, Raymond and Cestan, Mills, Rogues and Sirol, Posey). 

Convergence was impaired in fifteen cases (Parinaud (2), Sharkey, 
Sauvineau, Teillais, Poulard, Gordinier, Raymond and Cestan, Korni- 
low, Basevi, Cases 2, 4, 5, 8, and 9 of my series), and normal, or 
nearly normal, in nine cases (Hope, Parinaud, Nogues and Sirol, Kor- 
nilow, Posey, Cases 1, 3, 6, and 7 of my series). 

Ptosis was found in seven cases (Reinhold, Eisenlohr, Gordinier, 
Kornilow, Nothnagel, Cases 2 and 8 of my series), and was said to 
be absent in thirteen cases (Wernicke, Parinaud, Gee, Sauvineau, Teil- 
lais, Raymond and Cestan, Cases 1, 3, 4, 5, 6, 7, and 9 of my series). 

Necropsy was obtained in nineteen cases (Henoch, Wernicke, 
Gowers, Hope, Reinhold, Thomsen, Gee, Eisenlohr, Lichtheim (3), 
Sharkey, Hoesslin, Gordinier, Nothnagel, Basevi, Cases 1, 2, and 3 
of my series). 

In all of these cases except one (Thomsen 37 ) parts about the aque- 
duct of Sylvius were implicated, and in Thomsen's case a gumma was 
found in the cerebral peduncles. Thomsen cannot explain why the 
intense degeneration of the right oculomotor nerve and the slight de- 
generation of the left oculomotor nerve caused paralysis of only up- 
ward movement. It is possible that the Nissl method, if it had been 
employed, would have shown marked alteration of the oculomotor 
nuclei, possibly as a toxic effect from the adjacent gumma, and in this 
way the paralysis of upward associated movement may have been 
caused. Such was the conditon in one of my cases, unless perhaps the 
alteration of the nerve cells was caused by pressure. Until at least an- 
other case similar to Thomsen's is reported we may well hesitate to 
believe that a peripheral lesion of one oculomotor nerve may cause 
paralysis of only upward associated movements. 

Tumor was found in fourteen cases (Henoch, Gowers, Hope, Rein- 
hold, Gee, Lichtheim (3), Sharkey, Hoesslin, Gordinier, Nothnagel, 
Cases 1 and 2 of my series), a bullet wound in one case (Eisenlohr), 
apoplectic cyst in one case (Wernicke), hemorrhage in one case (my 
Case 3), and lesion uncertain in one case (Basevi). 

37 Archiv. fur Psychiatrie, vol. xviii. 1887, p. 616. 



TUMOKS AND OTHEE BRAIN LESIONS 417 

Recovery or partial recovery occurred in seven cases (Priestley 
Smith, Nieden, Verrey, Kornilow, Cases 5, 8, and 9 of my series). 
Important symptoms other than ocular palsies were found in forty-one 
cases (Henoch, Wernicke, Priestley Smith (2), Gowers, Parinaud (3), 
Hope, Reinhold, Thomsen, Nothnagel, Gee, Eisenlohr, Lichtheim (3), 
Verrey, Sharkey, Sauvineau, Hoesslin, Bruner, Teillais, Poulard, Gor- 
dinier, Cruzon-Marie and Babinski, Raymond and Cestan, Mills, 
Nogues and Sirol, Kornilow (2), Schroder, Posey, Parkinson, Cases 
1, 2, 3, 4, 6, 8, and 9 of my series). 

As a result of my studies I believe that persisting paralysis of asso- 
ciated lateral movement indicates a lesion of the posterior longitudinal 
bundle; that persistent paralysis of associated upward or downward 
movement indicates a lesion in the vicinity of the oculomotor nucleus, 
and that paralysis of associated ocular movements is not the result of 
a lesion of extracerebral nerve fibres. Lesions of the cerebral cortex 
may certainly cause paralysis of lateral associated ocular movements, 
and possibly of upward or downward associated ocular movements, but 
cortical paralysis of associated ocular movements is transitory, unless 
possibly where the centre on each side of the brain is destroyed. Paraly- 
sis of associated ocular movements may be caused by hysteria. Any 
case in which associated ocular palsy is persistent and is of organic 
nature, is unsuitable for operation unless the operation is merely pal- 
liative, as the lesion is probably within the posterior part of the pons 
or cerebral peduncle, according to the form of the associated palsy, or 
else causes much pressure upon the dorsal portions of these structures. 
The paralysis of associated ocular muscles may be produced by inflam- 
matory lesions or lesions of a similar character (alcohol, syphilis), as 
well as by tumor, and may disappear later in the course of the disease. 
Syphilitic ependymitis and cellular infiltration must be considered in 
diagnosing the lesion causing paralysis of associated ocular movements. 
Most congenital associated palsies are probably nuclear in origin. 

Drowsiness, Nystagmus, and Impairment of Vision as Signs of 
Tumor of the Corpora Quadrigemina. — Drowsiness and repeated 
yawning are not uncommon signs of a tumor of the corpora quadri- 
gemina, but are not pathognomonic. 

Nystagmus also occurs with tumor of the corpora quadrigemina and 
sometimes is obtained only by looking upward. 

A peculiar form of nystagmus is described by Bielschowsky as 

occurring in cases of tumor of the corpora quadrigemina. It consists 

of clonic movements of both internal rectus muscles, and seems to have 

been much the same form of movement as that observed by Ransom. 

27 



418 THE EYE AND NERVOUS SYSTEM 

Bielschowsky refers to Blanquinque as having described in 1871 con- 
vulsive movements of the eyeballs as a sign of tumor of the corpora 
quadrigemina. 

Vision may not be disturbed at all by a tumor of the corpora quad- 
rigemina, and when disturbed vision results from optic neuritis the 
latter probably is more frequently caused by internal hydrocephalus. 
Implication of the anterior colliculi of the corpora quadrigemina prob- 
ably does not cause marked disturbance of vision, but the external 
geniculate body of one side may be involved and give rise to hemianopsia 
if the tumor grows forward. 

In a case of tubercle of the corpora quadrigemina with tuberculous 
meningitis, reported by Nissen, the failure of vision seemed to be 
caused by the optic neuritis from meningitis, and not by the tubercle of 
the corpora quadrigemina. 

In W. Goldzieher's case of tubercle of the corpora quadrigemina 
the symptoms were: ataxic gait, drowsiness, complete paralysis of all 
external muscles supplied by each oculomotor nerve (the movements 
of the iris were normal and accommodation also seemed to be normal, 
as the child recognized small objects held near its eyes ; the eye- 
grounds were normal), weakness of the lower limbs, frequent yawning, 
sudden and severe involuntary jerkings of the whole body, etc. The 
child was five years old. 

The necropsy showed a tubercle of the corpora quadrigemina about 
the size of a hazel nut. The aqueduct of Sylvius was somewhat nar- 
rowed by pressure, but the gray matter about the ventricles did not 
appear to be implicated. 

The case shows, according to Goldzieher, that a tumor involving the 
corpora quadrigemina entirely may cause no blindness. He refers to 
Nothnagel as having spoken of this fact in his Topischen Diagnostik der 
Gehirnkrankheiten, pp. 211-214. Goldzieher's patient saw well, and 
accommodated. 

In Bielschowsky's case the anterior portion of the corpora quadri- 
gemina was completely degenerated, and each pulvinar was much com- 
pressed, but vision was only a little impaired, and the visual fields were 
not contracted. The external geniculate bodies were preserved, and to 
the integrity of these structures was due the preservation of vision. 

In some cases of tumor of the corpora quadrigemina the abducens 
nerve has been paralyzed, as in a case observed by W. B. Ransom, 38 in 
which there was a large oval-celled sarcoma of the corpora quadrigemina. 



Lancet, May 4, 1805, p. 1115. 



TUMOKS AKD OTHEE BKAIN LESIONS 419 

Ophthalmoplegia was well developed long before marked ataxia set in. 
In the earlier stage the diagnosis between growth from the base of the 
skull, of the corpora quadrigemina or pineal gland, and of the cere- 
bellum, was not certain. Paralysis of the sixth nerves, loss of knee- 
jerks, clonic spasm of the upper lids and internal rectus muscles, espe- 
cially of the left, were observed. The disturbance of vision in this case 
was regarded by Ransom as unusual, as in most cases such loss has been 
slight or very late in appearing. It could not in his case have been due, 
he says, to implication of the optic thalamus or optic radiations. It is 
possible that the optic neuritis caused it, but a similar amount of 
effusion is frequently seen with little or no loss of vision. The 
paralysis of each external rectus is interesting as the pons was normal. 
An explanation for this paralysis is not given, but the case is another 
instance of the paralysis of the abducent nerve when the lesion is at a 
distance from its nucleus. 

Optic neuritis was absent in a case of tumor in the corpora quadri- 
gemina observed by Frederick Taylor, 39 in which the cerebellum was 
perfectly healthy, but the corpora quadrigemina were flattened, broader, 
and gray and gelatinous in appearance, and were the seat of a new 
growth, regarded as a glio-sarcoma. The symptoms were : ptosis, stag- 
gering gait, drowsiness, ataxia of the upper limbs, nearly complete 
double ophthalmoplegia, lateral nystagmus and later unconsciousness. 
The pupils were slightly and unequally dilated and reacted to light, 
but the child could not see. 

Nissen 40 has made the peculiar observation that tumor of the brain 
in children is less likely to cause choked disks than is tumor in adults. 
In his five cases of tumor of the corpora quadrigemina or cerebral 
peduncle, optic neuritis was present only in one, and in this case there 
was meningitis. Xissen mentions as cases of tumor of the corpora quad- 
rigemina with normal eyegrounds those of Taylor, Goldzieher, Brims, 
and Ilberg. 

Resemblance between the Symptoms of Tumor of the Corpora 
Quadrigemina and those of Multiple Sclerosis. — The symptoms of 
tumor of the corpora quadrigemina may resemble closely those of mul- 
tiple sclerosis, and the differential diagnosis may be exceedingly diffi- 
cult. For example, in Bielschowsky's case 41 the temporal side of each 
disk was pale as in multiple sclerosis. This pallor was explained as 
secondary atrophy from pressure on the chiasm by the distended third 

39 Taylor, Lancet, November IS, 1893, vol. ii. p. 1252. 

40 W. Nissen Jahrbuch fur Kinderheilkunde, 1901, vol. liv. 

41 Bielschowsky, Deutsche Zeitschrift fur Nervenheilkunde. xxii. 1 and 2. p. 54. 



420 



THE EYE AND NERVOUS SYSTEM 



ventricle. Pressure on the chiasm may cause atrophy without first pro- 
ducing choked disks. This temporal pallor of the disks is confusing, 
especially in cases of tumor of the corpora quadrigemina, as Bielschow- 
sky points out, because incoordination is also present, and the diagnosis 
of multiple sclerosis is likely to be made. 

Nystagmus and coarse intention tremor in Gordinier's case were sug- 
gestive of multiple sclerosis, but Gordinier believed that severe head- 
ache, optic neuritis, ophthalmoplegia, and the cerebellar gait, together 




Fig. 11.— Tumor of orbit causing ptosis of left upper eyelid, almost immobile pupil, complete paral- 
ysis of superior rectus and inferior rectus, partial paralysis of internal rectus, some protrusion of eye- 
ball, and blurring of optic disk on the left side. The photograph shows an attempt to look to the right. 
The only sign of intracranial disturbance was exaggeration of the patellar reflexes. Had the tumor 
grown more within the cranium, the limbs of the right side would have been paralyzed in association 
with the left oculomotor paralysis. 



with the absence of scanning speech, of epileptiform attacks, and of 
evidence of motor-tract involvement, negatived such a diagnosis. This 
opinion can not be accepted unconditionally. To my mind the absence 
of involvement of the motor tract was the best clinical evidence that 
the disease was not multiple sclerosis. Headache, optic neuritis, cere- 



TUMORS AXD OTHER BRAIX LESIOXS 



421 



bellar gait may all occur in multiple sclerosis, and I have shown by a 
case with necropsy that ophthalmoplegia may also be a sign of the 
disease. Scanning speech and epileptiform attacks are not present in 
every case of multiple sclerosis. 

Symptoms from Implication of the Cerebral Peduncle. — AYhen the 
tumor extends far enough ventrally to involve the foot of the cerebral 
peduncle it may cause paresis or paralysis of the limbs on the side 
opposite to the lesion, with involvement of the oculomotor nerve on 



^ — '-i^^ffinn^HH 








Fig. 12.— Same patient as shown in Fi< 



11. Complete left-sided ptosis. (Case seen by author with 
Dr. S. D. Risley.) 



the same side as the lesion. Various forms of tremor may be present 
in the paretic limbs, the involuntary movements may have the charac- 
teristics of intention tremor, or may be choreiform or athetotic. 

In a case that B. Sachs 42 reports, one of thrombosis of the posterior 
cerebral artery, the right crus was small and very soft, and the right 
oculomotor nerve was as thin as a thread. Left hemiplegia had been 
present. The left upper limb had shown the wildest ataxic movements, 



"Sachs, Amer. Jour, of the Med. Sci., 1891, vol. ci. p. 233. 



422 THE EYE AND NERVOUS SYSTEM 

reminding one of the extreme incoordination of the lower limbs in 
the last stages of tabes dorsalis. If the patient were asked to use the 
paralyzed hand the paretic limb was whirled about so violently as to be 
a source of some danger to those near. After a little, the arm would fall 
down from exhaustion, and remain quiet until aroused again. 

In a case observed by Bristowe, 43 all the limbs were weak but not 
paralyzed, and were tremulous on movement; this, according to Bris- 
towe, seemed to indicate compression and irritation of the motor tracts. 

In another case a tuberculous growth occupied the third ventricle 
and implicated both optic thalami. Tremors like those of disseminated 
sclerosis and drowsiness were present in this case. 

Kolisch 4i says (1893) that ataxic gait has been observed in two- 
thirds of the cases of tumor of the corpora quadrigemina. Peculiar 
persisting choreiform movements of the left upper and lower extremi- 
ties, but ceasing during sleep, were observed even when his patient, a 
child, was lying on her back, and were more intense when she was ex- 
cited, sat up, or attempted to walk. These movements were attributed 
by Kolisch in his case to lesion of the tegmentum of the cerebral 
peduncle and irritation of the pyramidal tract. 

Gordinier says that in five of the eleven cases in literature (Bruns, 
Weinland, Eisenlohr, Ilberg, BonhofTer) in which lesions of the corpora 
quadrigeminal region were accompanied by tremor or choreiform move- 
ments, the superior cerebellar peduncles were involved. Both Bruns and 
Weinland attribute the tremor to irritation of the motor fibres of the 
pyramidal tracts, although, as Gordinier mentions, in the cases they 
report the necropsies showed the motor tracts to be normal, while the 
superior cerebellar peduncles were diseased. Gordinier refers to the ex- 
periments of Ferrier and Turner, who described similar tremors result- 
ing from division of the superior cerebellar peduncles in monkeys. They 
found that if a peduncle were divided between the cerebellum and its 
decussation in the tegmentum, the tremor was confined to the side of 
the lesion. I have referred in my section on tumors of the optic thala- 
mus to involuntary movements caused by implication of the superior 
cerebellar peduncles. 

Symptom-Complex of Benedickt. — Paralysis of the oculomotor 
nerve on one side and of the limbs on the opposite side, with tremor 
of any variety, has been called the symptom-complex of Benedickt, but 
is hardly to be expected in a typical form in tumor of the corpora 
quadrigemina, as the ocular nuclei are situated so close together that 

43 Bristowe, Brain, vol. vi. p. 167. 

44 R. Kolich, Deutsche Zeitschrift fiir Nervenheilkunde, vol. iv. p. 14. 



TUMORS AND OTHER BRAIN LESIONS 423 

the paralysis of the ocular muscles is usually bilateral, although uot 
commonly symmetrical. In order to have paralysis of the limbs the 
tumor must in some way affect the motor fibres in the foot of the 
cerebral peduncle. 

In a very interesting case of tubercle of the right cerebral peduncle, 
reported by Blocq and Marinesco, 45 there were symptoms like those of 
paralysis agitans. The diagnosis of paralysis agitans was made during 
the life of the patient. The immobile face was like that occurring in 
this disease. A rhythmical tremor of small oscillations was seen in the 
left hand, the left fingers were half flexed, the left lower limb trembled 
also but less severely. Voluntary effort arrested the tremor temporarily. 
The authors believe that this was not paralysis agitans occurring in a 
case of tumor of the cerebral peduncle, because of the exaggeration of 
the reflexes on the left side and the exact correlation of the symptoms 
with the localization of the tumor. Exaggeration of tendon reflexes ; 
however, does occur in paralysis agitans. 

They refer to a case of Mendel in which intention tremor of the right 
arm was found associated with a tubercle of the left cerebral peduncle. 
Charcot observed a case in which tremor like that of Parkinson's disease, 
with typical attitude of one of the upper limbs, was associated with a 
tumor compressing one of the cerebral peduncles. 

Deafness as a Sign of Lesion of the Corpora Quadrigemina. — 
Weinland 46 has made an important study concerning the effect of 
lesion of the posterior colliculi of the corpora quadrigemina upon hear- 
ing. In nineteen cases of tumor of the corpora quadrigemina disturb- 
ance of hearing was observed in nine cases, — i.e., in about half of the 
cases (Duffin, Klebs-Eischel, Gowers, Ferrier, Eischer, Hope, Noth- 
nagel, Ruel, Weinland). Disturbance of hearing was observed in four 
cases out of nine of some other form of lesion of the corpora quadri- 
gemina (Hintz, Reinhold, Henschen, Zenner). In five of the nine cases 
of tumor of this region, with disturbance of hearing, the deafness 
was bilateral, although not always equal on the two sides. In three 
cases (Ferrier, Ruel, Weinland) it was shown that diseases of one pos- 
terior colliculus causes disturbance of hearing on the opposite side. 
This is explained by the course of the fibres concerned in hearing. 
Fibres connecting with the cochlear nerve form the lateral fillet, which 
passes to the posterior colliculi of the corpora quadrigemina ; from 
here other fibres pass to the internal geniculate body and the first and 
possibly the second temporal convolutions. 

45 Blocq and Marinesco, Memories de la Soc. de Biologic 1893. p. 105. 

46 Weinland, Archiv. fur Psych., 1894, vol. xxvi. p. 353. 



424 THE EYE AND NEKVOUS SYSTEM 

Weinland shows that the cases of tumor of the corpora quadrigemina, 
in which deafness was not observed, do not lessen the importance of the 
posterior colliculi in the function of hearing. 

In Gordinier's case 47 hearing was normal although the posterior 
colliculi of the corpora quadrigemina were almost destroyed. The 
patient heard ordinary conversation distinctly and the watch three 
feet away from each ear. This was explained by the absence of impli- 
cation of the lateral fillet of either side or of the nucleus about which 
its fibres terminate. In Bielschowsky's case also the posterior col- 
liculi of the corpora quadrigemina were much compressed, and yet 
hearing was not positively affected thereby, so far as could be deter- 
mined, although the patient felt as though the hearing had become less 
acute. 

Diagnosis between Lesions of the Corpora Quadrigemina and 
those of the Cerebellum. — Extension of the tumor forward so as to 
involve the visual system, either the optic tract, external geniculate 
body, or the optic thalamus, causes homonymous lateral hemianopsia. 
Extension backward into the pons may result in paralysis of the motor 
and sensory portions of the trigeminal nerve, and of the facial and 
abducens. 

L. Bruns 48 emphasizes the difficulty in diagnosing between tumors 
of the corpora quadrigemina and those of the cerebellum, as ataxia and 
ophthalmoplegia may be caused by a tumor in either location. A tumor 
of the vermis is very likely to cause cerebellar ataxia, and by pressure 
upon the corpora quadrigemina may cause ocular palsies. 

He believes on theoretical grounds it is possible that a tumor else- 
where within the brain, for example in the frontal lobe, may cause 
ataxia with paralysis of ocular muscles. Where the symptoms begin 
with ataxia Bruns thinks the tumor is probably in the cerebellum ; where 
they begin with ophthalmoplegia the tumor is probably in the corpora 
quadrigemina. This is entirely contrary to Eothnagel's teaching, as the 
latter regards ataxia occurring as the first symptom before the ophthal- 
moplegia as especially diagnostic of tumor of the corpora quadrigemina. 
Bruns mentions as a diagnostic point that the escape of the abducent 
nerves would be more in favor of tumor of the corpora quadrigemina 
than of tumor of the cerebellum, but paralysis of these nerves may occur 
with tumor in either place. Implication of other cranial nerves, as of 
the facial, would indicate rather tumor of the vermis. Ataxia or chorei- 

47 Gordinier, Journal of Nervous and Mental Disease, October, 1901, p. 543. 

48 Bruns, Die Geschwiilste des Nervensystems, Archiv. fiir Psych., 1894, vol. 
xxvi. p. 299. 



TUMORS AND OTHER BRAIN LESIOXS 425 

form movements would indicate rather tumor of the corpora quadri- 
gemina. 

Nissen says that Xothnagel believed the posterior colliculi of the 
corpora quadrigemina are concerned in co-ordination. Others believe 
the ataxia is caused by involvement of the red nucleus or superior cere- 
bellar peduncle ; still others attribute the ataxia to pressure on the cere- 
bellum. In Xissen's case there was no ataxia and a tubercle was con- 
fined to the corpora quadrigemina and implicated only the upper part 
of the tegmentum. There was also no disturbance of hearing, although 
the posterior colliculi of the corpora quadrigemina were implicated. 
Xissen thinks that the corpora quadrigemina may be a reflex area, 
in which the acoustic system is connected with the nuclei for ocular 
movements. He thinks also that at least in some of the cases of tumor 
of the posterior colliculi with deafness the tumor may have pressed 
upon the internal geniculate body, or may have caused the deafness by 
distant pressure upon the medulla oblongata and acoustic nerve. 

In this remarkable case of Xissen there was neither ataxia of limbs 
nor paralysis of the ocular nerves, and Xissen shows that the symptoms 
recognized by Xothnagel (ataxic gait and ophthalmoplegia) depend 
upon implication of the tegmentum. 

In two cases of tumor of the corpora quadrigemina observed by W. 
Aldren Turner, 49 staggering gait combined with diplopia and headache 
varying in position were the chief symptoms. In one case the inclina- 
tion was invariably backward, so that the patient was unable to stand 
or even to sit up in bed without support from behind. The ocular symp- 
toms consisted in paresis of the third and sixth nerves in one case, and 
defect in the upward and downward movements of the globes in the 
other. Optic neuritis was present in both cases. In one case the knee- 
jerks were lost and in the other they were diminished. Both of these 
cases showed ataxia of cerebellar character with impairment in the 
movements of the eyeballs. 

Persisting subnormal temperature was observed in a case of glioma 
of the mid-brain and left optic thalmus reported by Lloyd. 50 The 
paralysis was crossed, the left third nerve being involved, with right 
brachial monoplegia and right crural monoparesis. The patient had a 
remarkable course of subnormal temperature, the lowest point reached 
being 95.4°. The temperature was subnormal for twenty days, with 
two exceptions. 



49 Turner, Brain, 1898, vol. xxi. 

50 Lloyd, Med. News, January 30, 1892, p. 129. 



426 THE EYE AND NERVOUS SYSTEM 

The headache in tumor of the corpora quadrigemina is not so severe 
and frequent as in cerebellar tumor, according to Mssen, and is not so 
well localized in the occiput. Vomiting seems to be one of the early 
symptoms. Only in a few cases were forced positions and forced move- 
ments observed. 

LESIONS OF THE PINEAL GLAND. 

Oppenheim 51 is very skeptical regarding the relation of the pineal 
gland to the sexual organs. ITeubner, Slawzk, and Oestreich believe 
that early and excessive development of the penis or mammary gland 
occurs with tumor of the pineal gland. Sexual excitement and priapism 
are mentioned by Biancone, Gutzeit, and Ilenrot. Neumann also be- 
lieves a relation exists between the pineal gland and the male sexual 
apparatus. 

Tumors of the pineal gland, Oppenheim says, cause almost the 
same symptoms as tumors of the corpora quadrigemina, only in the 
former the oculomotor, trochlear, and abducent nerves are not so often 
affected, but nystagmus is more common. 

In Zenner's case 52 of tumor of the pineal gland, rapid onset of blind- 
ness, deafness, blunted intelligence, without motor or sensory paralysis, 
were attribuated to acute internal hydrocephalus. The blindness was 
not believed to be due to optic neuritis, and the optic tracts appeared to 
be somewhat flattened. Vomiting, speech disturbances and difficulty in 
swallowing were attributed to pressure on the pons and medulla 
oblongata. 

TUMORS OF THE PITUITARY BODY. 

Tumors of the pituitary body not infrequently cause bitemporal 
hemianopsia, which later may give place to blindness. Choked disks 
may be associated with tumor of this part of the brain, but not infre- 
quently the optic neuritis is of mild grade, or there may be optic atrophy 
from the pressure on the chiasm without previous neuritis. Polyuria 
and polydipsia, even diabetes mellitus, have been observed as signs of 
such a growth. The tumor may develop very slowly. Acromegaly has 
been found so frequently in cases in which the pituitary body was en- 
larged that some relation between this disease and the alteration of the 
pituitary body probably exists. Sufficient cases, however, have been 
reported to show that tumor of the pituitary body does not always cause 
acromegaly. Bitemporal hemianopsia is a common sign of acromegaly. 

51 Die Geschwiilste des Gehirns, Oppenheim, p. 142 et seq. 

52 Philip Zenner, Alienist and Neurologist, 1892, vol. xiii. p. 470. 



TUMOKS AND OTHEE BKAIN LESIONS 427 

If a tumor at the chiasm grows to sufficient size it may implicate the 
ocular nerves in its neighborhood and cause ocular palsies. Often the 
bitemporal hemianopsia is followed by complete blindness of the eye on 
the side of the greater development of the tumor and still later by com- 
plete blindness of the other eye. Sometimes when a tumor implicates 
one optic tract near the chiasm it causes homonymous lateral hemi- 
anopsia, followed by complete blindness in the eye on the same side as 
the tumor and increasing failure of sight in the other eye. This is 
caused by extension of the tumor to the inner side of the other optic 
tract. 

Bitemporal hemianopsia can be caused only by a lesion at the chiasm. 
Binasal hemianopsia, according to Harris, has been observed in tabes 




Fig. 13.— Adenoma of the pituitary body. Case of acromegaly reported by Dr. F. A. Packard, 
from a specimen in my collection. Necropsy by Dr. Cattell. (See also illustrations of this 
case in article by Dr. C W. Burr. ) 

and in one or two obscure cases probably as a result of symmetrical 
neuritis of the outer parts of the chiasm. He says further that homony- 
mous hemianopsia may occur in tabes, as he has seen in two cases with 
optic atrophy, and in multiple sclerosis also with optic atrophy, probably 
due to a patch of sclerosis affecting the optic tract. 

It is improbable that a lesion at the optic chiasm can cause nasal 
hemianopsia as shown by Wilbrand and Saenger, because the crossed 
and uncrossed fibres are mingled at the lateral part of the chiasm, but 
von Monakow says the lateral part of the chiasm contains chiefly un- 



428 THE EYE AND NEKVOUS SYSTEM 

crossed fibres. A lesion on the outer side of each optic nerve near the 
chiasm, or on the onter side of each optic tract, might cause binasal 
hemianopsia. This subject has recently been carefully studied by W. T. 
Shoemaker. 53 

The definition of hemianopsia he gives is " Half defect in the field 
of vision of each eye, symmetrically placed, and caused by a common 
intracranial lesion." He believes that binasal hemianopsia can never 
result from a lesion of the chiasm on account of the anatomical arrange- 
ment of the fibres, and that in most instances it is a symptom of optic 
nerve disease. In eighteen cases reported in the literature and in one 
of his own of binasal hemianopsia demonstrable inflammatory disease of 
the optic nerves was found in twelve cases. Shoemaker refers to five 
cases which seem to have exhibited true binasal hemianopsia (Lang and 
Beevor, Graefe, Daae, Herschel, Mooren's second case). 

Tumor growing from the pituitary body sometimes causes intense 
drowsiness. It was present in the case of acromegaly reported by A. F. 
Witmer from my clinic and also in the case of the same disease reported 
by F. A. Packard. F. W. Mott 54 says that in three cases of tumor of 
the third ventricle, which started probably from the pituitary body, and 
in which during life symptoms of such a growth were present, a condi- 
tion of drowsiness was observed and was so intense that these patients 
would fall asleep while eating their meals, and in two of the cases this 
phenomenon was so marked a feature as to be the most prominent and 
early objective symptom. 

Tumor in this situation I believe also may cause frequent yawning, 
but I have seen this sign when the tumor was in the cerebellum. 

I have seen the entire third ventricle filled by a tumor growing from 
the pituitary body in a case in which the symptoms of acromegaly were 
pronounced (case of F. A. Packard, Fig. 13). Other illustrations of 
Dr. Packard's case are given in Dr. Burr's article. 

TUMORS OF THE OPTIC NERVE. 

Sometimes a tumor occurs in the intracranial portion of the optic 
nerves, as in a remarkable case reported by A. Pick. 55 Braunschweig 
collected cases of tumor of the optic nerves and concluded that exoph- 
thalmos was never absent in such cases. Pick shows by the report of 
his own case that the word " never" should have been omitted. At the 



53 Shoemaker, New York Med. Journal and Philadelphia Med. Journal February 
4, 1905. 

64 Mott, Brain, 1898, vol. xxi. 

56 A Pick, Brain, 1901, vol. xxiv. p. 502. 




Fig. 14.— Paralysis of right upper and lower limbs and of right side of face ; paralysis of left muscles 
of mastication and of left side of tongue. Tongue deviated to the left when protruded. Diplopia in 
looking to the left. A lesion of the pons was probably present in this case. 




Fig. 15.— Same case as represented in Fig. 14. The lower jaw deviated toward the left when the 
mouth was opened because of the paralysis of the left muscles of mastication. The tongue deviated 
toward the right when within the mouth. Paralysis of left side of tongue. 







Fig. 16. — Paralysis of each external rectus and of the left muscles of mastication from a tubercle in 

the left half of the pons. 




- 18 S? SS %* 








47 



Fig. 17.— Same case as shown in Fig. 16. Tubercle of the left half of the pons. 






TUMOKS AKD OTHEE BEAIX LESIOXS 429 

necropsy in Pick's case the optic nerves appeared very gray, but were 
not diminished in size. The optic nerves, chiasm, and stump of the 
optic tract did not stain well by the Weigert-Pal method, and on super- 
ficial examination seemed to be merely atrophied; but more careful 
examination showed numerous cells among the atrophied nerve-fibres. 
The tumor was a myxosarcoma. Early failure of vision is to be ex- 
pected in tumors of the optic nerve, and the reflex pupillary response 
may be present at one time and absent at another. The latter sign may 
not always be present, and is caused probably, as Pick says, by the soft 
tumor tissue within the nerve and by varying degrees of vascular con- 
gestion. Pick's patient had hallucinations of sight, similar to those 
sometimes occurring in disease of the retina or optic nerves. 

TUMORS OF THE PONS AND MEDULLA OBLONGATA. 

Ataxia is generally very pronounced in tumors of the pons, because 
of implication of the cerebellar peduncles, but I have had a case in 
which a large tumor on the pons caused no ataxia. Many important 
structures are crowded together in the pons, and even a small growth may 
cause very striking symptoms. When the tumor is confined to one side 
of the pons, it may cause paralysis of motion in the muscles of masti- 
cation and loss of sensation in the face on the same side as the tumor, 
because of involvement of the trigeminal nerve; paralysis of the ex- 
ternal rectus and of the muscles of the face on the same side as the 
tumor, because of involvement of the abducent and facial nerves ; in 
some cases also when the tumor extends low into the pons, deafness on 
the same side from involvement of the acoustic nerve, or of the intra- 
pontile fibres belonging to the acoustic system. The tongue and limbs 
may be paralyzed on the opposite side because of implication of the 
central motor-fibres for the hypoglossus nucleus, and of those for the 
anterior horns of the cervical and lumbar region above their decussa- 
tion. Sensation may be impaired in the limbs of the opposite side of 
the body and even in the opposite side of the face, because of implication 
of the sensory fibres above their decussation. Occasionally the tongue 
is paralyzed on the same side as the muscles of mastication (Figs. 
14 and 15). 

In a case of tubercle of the pons observed by Dr. C. S. Potts and 
myself, the symptoms were weakness of the muscles of the left side of 
the face, excepting the orbicularis palpebrarum ; weakness of the 
muscles of mastication on the left side, and fibrillary tremors of these 
muscles ; weakness of the left external rectus, with the loss of power 
of associated movement of the eyes to the left, but with preservation of 



•130 



THE EYE AND NERVOUS SYSTEM 



the power of convergence; diminution of the appreciation of touch, 
pain, heat, and cold in the right upper and lower limbs, right side of 
the trunk and neck, occipital region and ear; loss of stereognostic per- 
ception and of the sense of position on the right side ; diminution of 
the appreciation of touch, pain, heat, and cold on the left side of the 
face and head ; slight loss of appreciation of heat and cold on the right 
side of the face; anaesthesia of the conjunctiva, mucous membrane of 
the nose, mouth, and tongue on the left side ; deafness in the left ear ; 
loss of taste in the left anterior half of the tongue, and ataxia of both 




Fig. 18— Photograph showing the tendency of the head to fall forward and to the left, and the pres- 
ence of left hemiplegia in a case of glioma of the right side of the pons (Figs. 5-8). 

lower limbs, especially of the right. The tumor was confined to the left 
half of the pons. 

I believe that this case shows that paralysis of lateral associated 
movement, when the power of convergence is preserved, is caused by a 
lesion of the posterior longitudinal bundle between the nuclus of the 
sixth nerve and that of the third. I have since the publication of this 
case studied two other cases of tumor within the pons with necropsy, 
in which the correct diagnosis could be made during the life of the 
patient. In these cases the symptoms were similar to those described 
above, and in each case I was able to observe paralysis of associated ocu- 
lar movements toward the side of the tumor. 



TUMORS OF THE CEREBELLUM. 

Marcus Gunn 5G believes that intense double optic neuritis, with 
much swelling and surrounding retinal change, coming on quickly, sug- 
"(«unn, Brain, 1808, vol. xxi. 




Fig. 19 —Glioma of the right side of the pons. F G. = the raphe of the pons. The right portion of 
the tumor is cystic. Same case represented in Fig. 18. 



V 







Fig. 20.— Cyst and glioma of the vermis. 



1 -^ 




Fig. 21.— Position of standing in lesions of the cerebellum. 



TUMORS AND OTHER BRAIN LESIONS 431 

gests the cerebellum ; and that one-sided optic neuritis, or marked dif- 
ference in the intensity of the neuritis on the two sides, suggests the 
cerebrum, and is, on the whole, in favor of the tumor being on the same 
side as the excess of neuritis, where there are other reasons for localizing 
a tumor in the front of the cerebrum. 

Cerebellar tumor, he says, very often excites a peculiarly intense 
optic neuritis, with great engorgement of the papillae and marked oedema 
of the surrounding retina, with, not uncommonly, a macular stellate 
figure similar to what is seen in so-called albuminuric retinitis. This 
condition is seen also in basal meningitis, and possibly the reason for 
its occurrence with cerebellar tumor is that the latter is likely to cause 
basal meningitis. Mr. Gunn says he has found these changes in the 
eye-grounds in at least two cases of frontal tumor, in neither of which 
was there any evidence of basal meningitis. 

The changes resembling those of albuminuric retinitis were present 
in a number of cases of tumor that have come under my observation. 

Tumor of the cerebellum is often in connection with a cyst 
(Fig. 20), but the tumor may be so small that it may be overlooked, 
unless careful microscopical study is made ; indeed, some writers be- 
lieve that a cyst of the cerebellum is always at some time in its growth 
in connection with a tumor. 

The ataxia of cerebellar tumor is often intense, and the patient 
staggers from side to side like a drunken person (Fig. 21). Choked 
disks are generally pronounced. The headache may be occipital or 
frontal, and in some cases may be associated with rigidity of the neck. 
A tendency for the head and trunk to incline forward and to one side 
(Fig. 18) is pronounced in some cases, but it is uncertain whether 
the inclination of head and trunk is toward the side of the tumor or 
away from it. Hemiasynergy is an unreliable sign, although I have 
seen it in three or four cases. It may be tested by having the patient 
while lying down draw up each lower limb separately, and it should be 
observed then whether the leg is extended on the thigh and the thigh 
on the trunk synchronously, or whether there is a dissociation of these 
movements. The latter is supposed to indicate the presence of a lesion 
in the lateral lobe of the cerebellum on the side of the hemiasynergy. 

Vertigo is often an early and persistent sign of tumor of the cere- 
bellum. 

Staggering in walking, like the inclination of the head and trunk, 
may be toward or away from the side of the tumor. 

Deafness, such as may be caused by involvement of the acoustic 
nerve may be a valuable sign pointing to the side of the cerebellum 



432 THE EYE AND NEKVOUS SYSTEM 

affected, and is likely to be present when the tumor is in the cerebello- 
pontile angle. 

In a case of cerebellar abscess I had the opportunity to observe that 
nystagmus was greater when the eyes were turned toward the side of 
the lesion than when they were turned away from it. I have not been 
able to make this observation in any other case, although the sign has 
been seen by others. 

Tenderness to pressure over one side of the occipital region, and 
increase or diminution of vertigo according as the patient lies on one or 
the other side of the body, are signs of uncertain value. 

The patellar reflex is frequently lost in cases of cerebellar tumor, but 
it may be exaggerated, or may vary in intensity from time to time. 




Fig. 22— Paralysis of each external rectus from a tumor in the fourth ventricle, implicating the 

abdueens nuclei. 

Lesions Simulating Tumor. — Other lesions of the brain may cause 
symptoms resembling very closely those of tumor, and chief among these 
is abscess. In most cases some purulent focus may be detected as the 
cause of the cerebral abscess, and most frequently it is disease of the 
middle ear. In other cases pus in the ethmoid or sphenoidal sinuses 
may be the starting point for cerebral abscess, or there may have been 
some injury to the bones of the skull. The symptoms of abscess are 
likely to develop more rapidly than do those of tumor and may be asso- 



TUMOKS AND OTHEE BKAIJST LESIONS 433 

ciated with fever, or subnormal temperature, chills, slowing of the pulse ; 
or the symptoms of meningitis may be added, — viz., transitory ocular 
palsies, rigidity of the neck, Kernig's sign, etc. Purulent meningitis 
often accompanies cerebral abscess. 

When the meningitis is in plaques a differential diagnosis from 
brain tumor may be impossible. This form of meningitis, described 
especially by the French writers, is more common in the motor area, and 
I have described one of the two cases reported in this country. In my 
patient the symptoms were precisely those of tumor of the parietal 
lobe. 

Hydrocephalus in often associated with tumor and I have seen it 
so mask the symptoms of a tumor on the pons that the latter could not 
be diagnosticated. Again, I have seen internal hydrocephalus without 
tumor cause the symptoms of a cerebellar neoplasm. 

Hemorrhage into the brain can be easily distinguished. The 
rapidity of onset and the course of the symptoms are unlike what is seen 
with brain tumor, but sometimes hemorrhage into a tumor occurs, and 
the diagnosis then is made chiefly by the sudden increase of symptoms. 

The differential diagnosis between general paralysis of the insane 
(chronic meningoencephalitis) and cerebral tumor usually is easy, but 
I have known the former to be regarded as tumor, and the danger of 
mistake is greater when the encephalitis is of rather rapid onset. Usu- 
ally, however, a correct diagnosis can be made. 

Aneurysms may give the symptoms of brain tumor. In some cases 
the stethoscope applied to the head may be of assistance, but I have, 
known a supposed tumor at the chiasm causing bitemporal hemianopsia 
to be in reality *an aneurysm in this region, and to present no signs 
characteristic of aneurysm. 



LAS 



CHAPTER X. 

BULBAR AND PSEUDOBULBAR DISEASES 

« 

By E. W. TAYLOR, A.M., M.D. 

The clinical significance of affections of the oblongata lies essen- 
tially in the fact that in this portion of the brain are gathered, in small 
compass, nerve mechanisms of vital importance. The hypoglossal nerve, 
and the group made up by the spinal accessory, vagus and glossopharyn- 
geal, are situated here, in close relationship to each other. Disease 
processes, small in extent, may therefore lead to most serious disorders 
of function, if localized in the oblongata, and particularly in its dorsal 
portion. Owing to the great functional importance of this region, there- 
fore, a separate consideration of its diseases is justified, although it 
should be borne in mind that a considerable number of the bulbar affec- 
tions are simply local expressions of more general processes, either 
spinal or cerebral, or involving higher levels of the brain stem. Eor 
example, bulbar lesions are often associated with tabes, poliomyelitis, 
progressive muscular atrophy, including amyotrophic lateral sclerosis, 
multiple sclerosis, or with so-called Landry's paralysis, diseases which 
are ordinarily included in the spinal category. On the other hand, 
bulbar symptoms are not infrequently combined with generalized cere- 
bral lesions, of which arteriosclerosis, paralytic dementia, and syphilis 
will serve as examples. More important for our present purpose is the 
association with encephalitis, and especially with superior polienceph- 
alitis, ophthalmoplegia, and other affections of cranial nerve nuclei 
beyond the immediate limits of the oblongata. 

As knowledge of pathological processes grows, it becomes in- 
creasingly evident that degenerative or inflammatory conditions are 
rarely sharply limited to one small area in the nervous system, although 
their clinical manifestations may point predominantly toward such 
localized involvement. This is somewhat strikingly true of the bulbar 
diseases, which, as already suggested, in many cases constitute merely 
the most conspicuous part of the clinical picture, owing to the highly 
important functional significance of this portion of the brain. It is 
still convenient, however, to use the accepted nomenclature and to de- 
scribe the bulbar lesions under a separate heading. 
434 






BULBAK AND PSEUDOBULBAK DISEASES 435 

General Symptomatology. — The symptoms arising from disease of 
the oblongata alone are chiefly dependent upon lesions involving the 
nuclei of the ninth, tenth, eleventh, and twelfth nerves. Through in- 
volvement of the vagus breathing is interfered with, leading to varying 
degrees of dyspnoea and at times to the Cheyne-Stokes type. The normal 
action of the heart may be disturbed, with irregularity of the pulse, or 
with an increased rapidity or abnormal slowness, depending upon the 
condition of paralysis or irritation of the heart regulatory mechanism. 
A more important and characteristic sign of bulbar injury is difficulty 
in deglutition, brought about by involvement of the accessory-vagus- 
glossopharyngeal group of nerves. Mastication may also suffer through 
disordered innervation of the hypoglossal nerve, and of the motor 
branches of the trigeminal to the jaw muscles. This latter difficulty is 
usually not conspicuous, since the degenerations in the motor nuclei 
of the fifth nerve lying in the pons are usually slight, if not entirely 
lacking. The part taken by the tongue in the act of mastication is, how- 
ever, sufficient in all well-marked cases to render imperfect the disposal 
of food in the mouth. Paralysis and atrophy of the tongue is one of 
the most constant disturbances in the course of bulbar disease. This 
leads to difficulty in forming a bolus of food, and in passing it backward, 
and also to a characteristic speech disturbance which leads very early to 
difficulties in articulation, progressing from an imperfect enunciation of 
certain sounds (dysarthria) to an ultimate complete disability to articu- 
late (anarthria). This is due not only to the paralysis of the tongue, 
but also to disturbances in the innervation of cheeks, lips, and palate, 
which determine the shape of the mouth cavity. Owing to difficulty of 
deglutition, as well as to probable disturbance in the centre in the oblon- 
gata, presiding over secretion, the saliva is apparently secreted in in- 
creased amount, and constantly flows from the imperfectly closed mouth. 
This combination of symptoms, more or less developed in various cases, 
constitutes the essential picture of the primary lesions of the oblongata. 

A fairly sharp distinction may, in general, be made between those 
more chronic lesions which are sharply limited to the oblongata, and 
others of more acute onset and more extensive distribution. In the latter 
the general symptomatology becomes much wider in range, owing to the 
fact that the lesions are no longer limited to the bulb, but extend to the 
pons, and even in certain cases to the mid-brain region of the corpora 
quadrigemina and crura cerebri. This widening of the scope of so- 
called bulbar diseases is not strictly accurate, but is justified and necessi- 
tated by the diffuse character of many of the underlying lesions, and on 
the clinical side by the frequent uncertainty of accurate focal diagnosis 



436 THE EYE AND KEKVOUS SYSTEM 

of disease involving the pons-oblongata region. In general, the symp- 
toms are dependent upon lesions of the nerves already mentioned, 
having their nuclei in the oblongata, and also upon the seventh, sixth, 
and fifth nerves located in the pons, together with the fourth and third 
nerves, lying in the quadrigeminal region. Paralysis of the face, dis- 
turbance in the innervation of the jaw, and various paralyses of the 
ocular muscles, are therefore frequent. Added to these are disorders of 
motility of the extremities from involvement of the ventrally lying 
pyramidal tracts, as well as varying alterations of sensibility, dependent 
upon interruption of sensory tracts. Except for the symptoms referable 
to lesions at a distance or of unknown cause, which will receive detailed 
discussion later, the symptomatology of this complicated region is 
wholly dependent upon a disturbance of function, either through irrita- 
tion or destruction of its component parts, or through the cutting off of 
its central connections. 

A distinction may in general be made between those affections which 
are degenerative in character and chronic in course, and those of acute, 
or sub-acute onset, in which the nerve elements are involved secondarily 
to vascular changes. Chronic progressive bulbar paralysis is a type of 
the first class, and so-called apoplectiform bulbar paralysis, brought 
about by embolism or thrombosis of arteries, a type of the second. A 
third general group includes those conditions in which the lesions 
apparently producing bulbar symptoms lie at a distance from the oblon- 
gata, — pseudobulbar palsy, — and finally those cases in which no lesions 
have been found sufficient to account for symptoms predominantly of 
bulbar type, — bulbar paralysis without anatomical basis ; myasthenia 
gravis in some of its forms. 

The following discussion will elucidate these more or less distinct 
processes, all of which have been differentiated within the past half 
century. 

PROGRESSIVE BULBAR PALSY. 

Terminology. — Various terms, suggestive of the location and of the 
course of this disease, have been used since the earliest description by 
Duchenne. The more important of these are : Progressive muscular 
paralysis of the tongue, palate, and lips (Duchenne) ; chronic pro- 
gressive bulbar paralysis (Wachsmuth) ; progressive nuclear bulbar 
paralysis (Kussmaul) ; labio-glosso-laryngeal paralysis (Trousseau) ; 
progressive amyotrophic bulbar paralysis (Leyden). 

History Following an observation of Dumesnil in 1859, in which the sug- 
gestion was made that bulbar lesions and progressive muscular atrophy might 



BULBAR AND PSEUDOBULBAR DISEASES 437 

co-exist, Duchenne 1 in 1860-61 gave the first satisfactory description of bulbar 
paralysis. He critcised Dumesnil's observation on the ground that progressive 
bulbar paralysis and progressive (spinal) muscular atrophy were absolutely dis- 
tinct diseases, the first being characterized by paralysis without atrophy, and the 
second by atrophy without paralysis, a false assumption, as later demonstrated. 
Observations by Romberg and Trousseau, previous to Duchenne's description, did 
not indicate a close conception of the character or manifestations of the disease. 
In 1868 Trousseau, on the basis of cases with somewhat unsatisfactory autopsies, 
completed Duchenne's earlier description, but maintained the essential identity of 
the disease with progressive muscular atrophy. Microscopic examination revealed 
certain degenerations of the hypoglossal and accessory nerves, but threw no definite 
light on the changes in the nuclei. The investigations of Baerwinkel, Wachsmuth, 
and Schultz definitely increased knowledge of the subject in the years up to 1865. 
Baerwinkel was struck with the apparent retention of electrical reactions in 
affected muscles, and also with the sharp localization to bulbar nerves. Wachs- 
muth in 1864 described certain alterations in the oblongata, which were, in part, 
correctly brought into relation with the clinical symptoms. The pathological 
anatomy was placed on a firm basis by the work of Charcot and Joffroy in 1869, 
verified and carried further by Charcot's pupils, as well as by Leyden (1870). 
The degeneration of certain motor nuclei in the oblongata, with the associated 
and resultant alterations of nerves and muscles, was determined as the essential 
lesion. Kussmaul (1871) urged the neuropathic character of the degeneration and 
established the analogy to progressive (spinal) muscular atrophy on a firmer 
basis. Cases thereafter were rapidly reported, with increasingly accurate descrip- 
tions, both on the clinical and anatomical side, by Gombault, Duchenne, Joffroy, 
Hun, Dejerine, Freund, Richie, Reinhold, Hoffman, Tooth and Turner, Remak, Turner 
and Bullock, and many others. At the present time the affection and its relations 
are adequately understood. The work of the past fifteen or twenty years has been 
to differentiate from the chronic progressive type of bulbar paralysis other varieties 
of bulbar disease which will be considered in detail later in this chapter. 

Symptomatology. — Bulbar paralysis of the progressive type, both be- 
cause it was first described and also because it presents a most charac- 
teristic picture of bulbar disease in general, may serve as a type of 
lesions of this region. 

The symptoms, as observed in a typical case, are as follows: The 
onset is gradual, usually indicated by a slight disturbance of speech, to 
which small importance is at first attached. A persistence of this symp- 
tom, combined with a sensation of pressure about the neck, finally leads 
the patient to a realization of the abnormal condition. As in many 
forms of disease of insidious onset, a slight exacerbation of symptoms 
may give rise to the idea of a sudden onset, which in this affection may 
be assumed never actually to occur. With the progress of the disease 
the tongue is affected; its movements are hindered; subjective sensa- 
tions of weight or stretching in the tongue and throat are noticed, which 
may extend downward. In conjunction with these symptoms the speech 
becomes more difficult, more indistinct and slower, and accompanied by 

1 Duchenne: De l'electrisation localizee, 2d ed., p. 621, 1861; also Arch. Gen. 
de Med., xvi. 283, 1860. 



438 THE EYE AKD KEKVOUS SYSTEM 

feelings of fatigue. The indistinctness of utterance is brought about 
first by difficulty in the enunciation of certain letters, in general, in- 
versely to the process of learning to speak. The sounds " r" and " sch" 
are early lost, as in the child they are last to be acquired. Later, as the 
movements of the tongue become more and more hindered, enunciation 
of the lingual consonants " 1, r, n, and t," and afterwards " s," is lost 
(Gowers, Ley den, and Goldscheider, quoting Kussmaul, find that " s" 
is early lost) ; " t and d" are involved when the tongue can no longer 
be brought firmly against the palate. Succeeding or associated with the 
palsy of the tongue, the lips are involved, with the result that speech is 
still further hindered in the pronunciation of the labials, " o, u, p, b ? 
m." Even when the disorder has reached this stage words may still be 
correctly enunciated by special effort and deliberation. The vowel 
sound " a" is longest retained. Finally, with the advent of weakness of 
the palate, complete paralysis of the tongue and inability to use the lips, 
speech is reduced to a few meaningless sounds, which bear only a distant 
similarity to articulate language. 

Before this final stage is reached, difficulties in deglutition super- 
vene, due to the combined weakness of the tongue, soft palate and pha- 
ryngeal muscles. Eood can no longer be properly moved into the back 
of the mouth cavity by the tongue, and the act of swallowing is likewise 
difficult. Mastication also suffers from the palsy of the tongue. Liquids 
are regurgitated through the nose, and with the progressive weakness of 
the muscles of the epiglottis particles of solid food enter the larynx. 
Swallowing becomes increasingly difficult, and semisolids are found to 
be more readily taken than either solids or liquids, owing to the lesser 
likelihood of finding lodgement in the larynx or being regurgitated 
through the nose. Along with the difficulty in swallowing, saliva col- 
lects in quantity, and finally runs in a constant stream from the mouth, 
which offers small resistance on account of the palsy of the lips. The 
patient at this stage presents the characteristic picture of bulbar disease, 
practically speechless, swallowing with the utmost difficulty, and con- 
tinually wiping away saliva as it flows from the imperfectly closed lips. 

As the disease still further advances, the muscles governing the vocal 
cords are apt to suffer; paralysis of the cords results and phonation 
finally becomes impossible. Coughing ultimately becomes difficult, with 
the accompanying danger of inhalation pneumonia. With such a grad- 
ual increase of paralysis in tongue, lips, pharynx, and finally larynx, 
the disease runs its course. As Gowers puts it, " the symptoms are 
grouped about the tongue as a centre," but they extend far beyond the 
limits of this one organ, before life is actually threatened. 



BULBAK AND PSEUDOBULBAR DISEASES 439 

Other symptoms which occur, but do not dominate the typical clin- 
ical picture, should be mentioned. Although there is no loss of sensi- 
bility, and taste is usually unaffected, the throat reflexes ordinarily fail, 
owing to the motor involvement, though this is not invariably the case. 
This tendency to loss of reflex activity naturally renders deglutition 
more difficult and increases the danger of the entrance of food into the 
larynx. The jaw reflexes, on the contrary, may be increased, no doubt 
due to a degeneration of the pyramidal tracts. (See Pathological 
Anatomy.) Dyspnoea, rapid pulse, oedema of the lungs, have been 
attributed to late involvement of the motor vagus (accessory). Except 
for an undue emotionalism, laughter or tears from insufficient cause, 
the mental faculties remain unimpaired, and the patient is painfully 
conscious of his sufferings up to the very end of the disease. 

Physical Signs. — The physical signs of the affection are as unmis- 
takable and characteristic as its symptomatic course. The nerves in- 
volved are the hypoglossus, the motor portions of the vagus-glossopha- 
ryngeal-accessory group, rarely the motor portion of the trigeminus, the 
facial, and ocular nerves. The involvement of these latter nerves is 
rather to be regarded as a complication than as a component part of the 
symptom-complex, assuming, as we do somewhat arbitrarily, that pro- 
gressive bulbar paralysis is a disease sui generis, limited to the 
oblongata. As a direct result of the primary degeneration of the hypo- 
glossal nerve, the tongue atrophies; as the disease progresses, it be- 
comes wrinkled, and furrowed, often swollen in appearance, and lies 
practically immovable on the floor of the mouth. A similar atrophy 
occurs in the lips ; a complete closing of the mouth finally becomes im- 
possible, and the lower portion of the face assumes a characteristic ex- 
pressionless appearance in marked contrast to the usually alert and 
distressed aspect of the eyes and upper portion of the face. 

The fact that the upper distribution of the seventh nerve is spared, 
whereas the lower branches are apparently involved in the degenerative 
process, has led to considerable speculation as to the course of the lower 
fibres, with the general conclusion, as just stated, that the orbicularis 
oris is supplied either directly or indirectly by fibres of the hypoglossal 
nerve, but connected with the facial through the dorsal longitudinal 
fasciculus. The close physiological association of lips and tongue in 
phonation renders probable the assumption that the innervation is from 
a common source, or from areas in intimate anatomical relationship. It 
is, at least, certain that atrophy of the lip muscles is a constant feature 
of progressive bulbar palsy, whereas, in exceptional instances only are 
the upper facial muscles involved. In certain cases of bulbar paralysis, 



440 



THE EYE AND NERVOUS SYSTEM 



however, forming part of a general amyotrophic lateral sclerosis, the 
facial nerves may be involved in all branches ; in other cases, as reported 
by Remak, 2 Tooth and Turner, 3 Turner and Bullock, 4 and others, the 
evidence goes to show that in spite of nuclear and other degeneration of 
the seventh nerves, the upper branches have still been spared. Follow- 
ing an earlier suggestion of Mendel, 5 that the upper facial muscles were 
supplied by fibres of the third nerve, through the dorsal longitudinal 
fasciculus and the trunk of the facial nerve, Tooth and Turner were 



\ 



IfP^ 







|k ; 



Fig. 1.— Progressive bulbar paralysis, patient of 39, six months duration. Deglutition very difficult ; 
speech wholly impossible ; palsy of tongue, lips, and muscles of deglutition ; exaggerated deep reflexes 
The photograph shows an extreme attempt to open the mouth and protrude the tongue. 

able to demonstrate a complete atrophy of the nucleus of the seventh 
nerve and also of its dorsally directed fibres and the knee, whereas the 
outgoing nerve contained normal fibres. From this observation, and 
others of similar character, it has been assumed that the upper facial 
muscles derive their supply indirectly from the nucleus of the third 
nerve. This would explain the sparing of the upper branches in those 
cases of bulbar palsy in which the lower branches of the seventh nerve 
were manifestly involved without assuming the intervention of the 
hypoglossal. It would also fall in with the physiological hypothesis, 
as already suggested, that muscles acting in association have a closely 



2 Remak: Arch. f. Psych., xxiii., H. 3, 919, 1892; Neurol. Ctb., vii. 62, 1888: 
idem, x. 28, 1891; idem, xi. 753, 1892; Berl. klin. Woch., xxxii. 29, 1895. 
3 Tooth and Turner: Brain, xiv. 473, 1891. 
"Turner and Bullock: Brain, xvii. 693, 1894. 
5 Mendel: Berl. klin. Woch., xxiv. 913, 1887; Neurol. Ctb., vi. 537, 1887. 



BITLBAK AND PSEUDOBULBAR DISEASES 441 

related nerve supply, — lips and tongue in articulation, opening and 
closing of the lids through the action of the levator palpebral (third 
nerve) and the orbicularis palpebrarum (seventh nerve, through fibres 
derived from the third nucleus). 

On the other hand, Wilbrand and Saenger 6 are of the opinion that 
the nucleus of the third nerve has nothing whatever to do with muscles 
supplied by the upper facial. In support of this opinion they quote a 
careful examination by Cassirer and SchifT in which there was no dis- 
turbance in the function of the upper facial in spite of complete de- 
generation of the oculomotor nucleus, including its dorsal portion, to 
which Mendel and others had attached particular significance. Siemer- 
ling and Boedecker also oppose the idea that the facial distribution to 
the eye muscles has any relation whatever with the nucleus of the third 
nerve. Evidence is, therefore, as yet conflicting as to the course of the 
fibres innervating the upper portion of the face, although the fact must 
be accepted that in nearly all cases of chronic bulbar paralysis the lower 
facial muscles are alone involved. The possibility of at least a partial 
nerve supply from the region of the hypoglossal nucleus for these 
muscles must be considered, as already suggested. 

In those cases of otherwise uncomplicated bulbar paralysis, in which 
the upper branches of the seventh nerve have been involved in the 
paralysis, it is probable that we have to do with a family type of the 
disease, as observed by Londe 7 and Remak. 8 Londe found in a child 
bulbar paralysis, with general involvement of the upper branches of the 
facial nerve, together with palsy of the levator palpebral superioris, 
but without other ophthalmoplegia. Remak has also reported a number 
of cases of involvement of the upper facial muscles in children, which 
led to the suggestion of a special type of family bulbar paralysis, having 
analogies to, but not identical with, the adult form. 

In general, regarding the greater or less involvement of the upper 
cranial nerves, lying in the pons or above, and including the facial 
nerve, it is now sufficiently evident that the underlying process is by no 
means a sharply limited one, but that, as already suggested, it may 
extend both upward and downward from its point of earliest develop- 
ment in the nuclei of the bulb proper. This fact will receive further 
comment in the discussion of the pathological anatomy. 

Electrical Reactions. — The electrical alterations in bulbar paralysis, 



Wilbrand and Saenger: Die Neurologie des Auges, Bd. I., Abt. 1, p. 114. 
Londe: Rev. de Med., xiii. 1020, 1893; xiv. 212, 1894. 
Remak: loc. cit. 



442 THE EYE AND KEKVOUS SYSTEM 

as in other analogous affections, depend upon degenerative changes in 
the peripheral motor neurones with consequent muscular atrophy. The 
early confusion inaugurated by Duchenne, and later set at rest by 
Trousseau, Kussmaul and all later observers regarding atrophy and 
paralysis, and the relations of bulbar palsy to progressive muscular 
atrophy, was enhanced by the apparent failure of electrical changes in 
the tongue. It is, however, undoubtedly true that if the muscles of the 
tongue could be stimulated with the same ease as other muscles, elec- 
trical alterations of greater or less degree would always be found. The 
deposition of fat in the atrophying tongue is one of the chief hindrances. 
Observations hitherto made have not demonstrated a complete reaction 
of degeneration, but partial degenerative reaction (Erb), and various 
stages between that and the normal have been observed so frequently 
as to substantiate the a priori assumption of electrical alterations of 
some degree in all cases in which the hypoglossal nerve is degenerated. 
Paralysis of the tongue and neighboring parts from other lesions than 
those affecting the peripheral motor neurones naturally would not give 
rise to electrical changes, and no doubt it is these cases which have led 
to the confusion. 

Duchenne and later Dejerine 9 have drawn attention to the fact that 
the paralytic symptoms of bulbar paralysis may be explained by de- 
generation of the pyramidal tracts. To this may be associated degenera- 
tion of peripheral neurones, giving rise to a bulbar amyotrophic lateral 
sclerosis, or an amyotrophic lateral sclerosis of descending course. A 
paralytic and atrophic form have been distinguished. It is in general 
evident that the atrophic element, with altered electrical reactions, will 
predominate when the peripheral neurones are involved ; that the spastic 
element will be conspicuous if the central neurones (pyramidal tracts) 
are involved, and that transitional forms may occur with varying de- 
grees of degeneration of the two sets of neurones. The analogy is com- 
plete to spinal progressive muscular atrophy of the flaccid and spastic 
(amyotrophic lateral sclerosis) types respectively. Finally it should 
be remembered that progressive bulbar paralysis, if not too quickly 
fatal, represents either the starting of a degenerative process, which 
tends to progress beyond the confines of the bulb, or else develops as the 
final stage of analogous affections originating in the upper spinal cord. 

Pathological Anatomy. — Progressive bulbar paralysis is characterized 
by a chronic, primary degeneration of the cells of the motor nuclei of 
the oblongata. This degeneration is not necessarily associated with 



Dejerine: Arch, de Physiol., xv. 180, 1883. 



BULBAR AND PSEUDOBULBAR DISEASES 443 

primary alterations of the vessels, nor with evidences of inflammatory 
reaction, and should be distinguished from those affections of the 
oblongata which are dependent upon vascular disorders or inflammatory 
processes. (See following sections.) The cells of the hypoglossal 
nuclei suffer most extensively; many of them show marked degenera- 
tive changes, are shrunken or entirely disappear. The accessory hypo- 
glossal nuclei are spared. Less marked, but still evident, are degenera- 
tions in the motor nuclei of the accessory nerve and of the cells of origin 
of the motor vagoglossopharyngeal roots. Degenerations have at times 
been found in the nucleus ambiguus, also in the nuclei of the seventh 



■: m 



":.v 








Fig. 2.— Progressive bulbar paralysis ; nucleus of hypoglossal nerve in centre of field, showing loss 
of myelinated fibres and almost complete disappearance of nerve cell bodies; x 45. (Photograph by 
W. S. Greene.) 

pair and motor nucleus of the fifth nerve in those not infrequent cases 
in which the disease progresses beyond the oblongata into the pons. Un- 
less we regard the dorsal nucleus of the vagus and glossopharyngeus as 
motor, at least in part, 10 it is difficult to account for the paralyses of 
larynx and pharynx, in those cases in which the nucleus ambiguus has 
been found intact, unless their supply be wholly through the accessory 
nerve. These matters require further investigation on the pathological- 
anatomical side. 

Corresponding to these cell degenerations the hypoglossal network of 



Spiller: Univ. of Penna. Med. Bulletin, March, 1903. 



444 



THE EYE AND NEKVOUS SYSTEM 



myelinated fibres is degenerated, and the intra- and extra-bulbar axones 
of the affected cells. The ensuing muscular atrophy shows no peculiar- 
ity. The muscle-bundles, particularly of the tongue, are thinned, the 
connective tissue increased, granular and fatty degeneration occur often 
with an excessive deposit of fat, which may somewhat mask the atrophy. 
The tip of the tongue suffers most. 

A very frequent, though not absolutely constant, accompaniment of 
the muscular alterations ,is degeneration of the pyramidal tracts. 
Opinions differ as to the frequency of this association. A number of 
cases have been described in which no alterations in the pyramidal 
tracts were found; they may, nevertheless, have been present in very 



::. -^ 




Fig. 3.— Progressive bulbar paralysis; tongue; atrophy of muscle bundles; increase of fat; x 45. 

(Photograph by W. S. Greene.) 

slight degree, as might have been shown had the Marchi method been 
used. It has been suggested (Kemak) that rapidly fatal cases are less 
likely to show degeneration of the pyramidal tracts, but that such de- 
generation would .probably have developed had life been prolonged. 
This question is precisely analogous to that long discussed with regard 
to progressive muscular atrophy with or without degeneration of the 
pyramidal tracts. In general, the evidence goes to show that the 
peripheral neurones may be degenerated independently of the central, 
but that the converse very rarely occurs. 

Etiology, Course, and Treatment. — The cause of the affection is wholly 
unknown. Syphilis and alcohol have no direct bearing on its develop- 



BULBAK A1STD PSEUDOBULBAR DISEASES 445 

ment. It occurs more frequently among men than women — 34 men 
and 19 women in a series of 53, as observed by Kussmaul. Apart from 
the rare, apparently congenital type, observed in children, it is dis- 
tinctly a disorder of the latter years of life, most frequent between the 
fiftieth and the seventieth years. I have, however, recently observed 
two cases in men, each thirty-nine years old. 

The course is from one to three years, and in certain cases much 
longer. In general, it is steadily progressive and death results from 
paralysis of respiration and deglutition, or from inhalation pneumonia 
in consequence of pharyngeal paralysis. The prognosis is, therefore, 
wholly bad, although occasional remissions may occur. 

The treatment must be directed towards proper diet, especially 
in the later stages. Semisolid food is usually indicated, and when 
swallowing becomes too difficult or impossible, the stomach tube becomes 
a necessity. General hygienic measures are essential, and encourage- 
ment serves better than drugs. Strychnia in increasing doses may be 
used ; its efficiency is doubtful. Kussmaul and later Remak have urged 
the value of the galvanic current for the purpose of stimulating the 
muscles concerned in swallowing. Remak advises that the anode be 
placed at the back of the neck, and the cathode passed over the neck 
on either side of the trachea, using a current of 3-6 milliamperes. 

ACUTE (APOPLECTIFORM) BULBAR PARALYSIS. 

Hemorrhage, Embolism, Thrombosis of Arteries of Bulb and Pons 

Under this general heading may be included those affections of the 
oblongata and pons, which are characterized by relatively sudden onset, 
and are either rapidly fatal or else show a tendency towards improve- 
ment. They are to be distinguished from the foregoing progressive 
bulbar paralysis on the clinical side by their non-progressive course, and 
on the anatomical side by their wholly different pathological altera- 
tions. 11 

Inasmuch as the lesions producing the symptoms of acute bulbar 
paralysis are mainly dependent upon the blood supply of the oblongata- 
pons region, the following anatomical facts should be borne in mind: 
The two vertebral arteries unite to form the basilar artery at the lower 
border of the pons, having given off, among others which do not now 



11 Van Voort (Deuts. Ztschft. f. Nervenheilk., viii. 137, 1896) has published a 
valuable discussion, with an elaborate table, on Acute Bulbar Paralysis, and very 
recently Dana (Trans. Ass'n American Physicians, xviii. 446, 1903) has published 
an excellent and comprehensive paper on the same subject. 



446 



THE EYE AND NERVOUS SYSTEM 



concern us, branches to the oblongata. The basilar artery before di- 
viding into the posterior cerebral arteries gives off transverse branches 
to the pons. These arteries run for a distance on the surface of the 
pons and then penetrate its substance. Erom studies made by Duret, 
it appears that the nuclei of the hypoglossal and accessory nerves receive 
their blood supply from the anterior spinal artery and the vertebrals; 
the nuclei of the vagus, glossopharyngeal and auditory from branches 
from the upper portion of the vertebrals, or lower part of the basilar; 
the facial, trigeminal, and ocular nerves from the branches of the 
basilar. Clinical experience, however, has not as yet justified complete 
reliance on these facts. 

Pathological Anatomy. — The lesions underlying a large proportion of 
the acute bulbar affections are alterations in the blood-vessels, which 




Fig. 4.— Acute (apoplectiform) bulbar paralysis; pons: lighter areas softening, as result of thrombosis, 
of basilar artery. (Photograph by W. S. Greene.) 



lead secondarily through weakness of their walls to hemorrhage, or 
through roughening or narrowing of the lumen to thrombosis, and when 
the proper conditions are present, to embolism. The sources of the 
vessel changes, and their consequent failure to carry on their normal 
function, are those recognized in arterial disease generally, — cardio- 
renal affections, syphilis, senility and the arteriosclerosis which & 
common in middle life and almost physiological in old age. Emboli 
may also reach this region from diseased heart valves. The basilar 
artery is a frequent seat of marked sclerotic change, and often shows a 



BULBAK AND PSEUDOBULBAK DISEASES 447 

tortuous course, irregularly thickened walls, and aneurisnial dilatations 
which may exert destructive pressure on the overlying oblongata. The 
same is true, though perhaps to less degree, of the vertebrals. The pons 
and oblongata must, therefore, both suffer from such lesions, since their 
immediate blood supply is wholly from these larger trunks. The soften- 
ings, or more rarely, hemorrhages, which may result, do not differ his- 
tologically from such lesions observed elsewhere in the brain, and 
require no detailed description here. The character of the blood supply 
naturally gives rise to the possibility of multiple lesions. The pons and 
oblongata, for example, are often simultaneously involved, and it is 
frequently impossible to localize the lesion accurately in one or the 
other region, hence the general inclusion of the symptoms under the 
head of bulbar paralysis. A higher level than the pons may also at 
times be involved, still further extending the inclusiveness of the term. 
Owing to the facts that the lesions, particularly of softening, may be 
multiple, that they are often extensive, and that they show no special 
predilection for the gray matter, it follows that the symptoms produced 
are far less definite than in the circumscribed chronic progressive form, 
and therefore that wide-spread disorders of motility and sensibility may 
result. 

BULBO-PONTINE HEMORRHAGE— ACUTE BULBAR APOPLEXY. 

Symptomatology. — Hemorrhage of the pons is rare ; it has been esti- 
mated that hemorrhage of the brain (cerebrum) is about fifty times as 
frequent. Hemorrhage of the oblongata is still less common. It occurs 
most frequently in the fourth decade of life, and is usually due, as in 
the brain, to the results of cardiac hypertrophy in the course of renal 
degeneration associated with arteriosclerosis. The branches of the 
basilar are the usual sites of rupture ; the basilar itself is rarely in- 
volved. 

The symptoms in a typically severe and fatal case, following in gen- 
eral Dana's description, are : Prodromal neck pain, headache, vertigo, 
vomiting, succeeded at varying intervals by an apoplectic seizure often 
of extreme suddenness. This is usually followed by profound coma, 
twitching of face and extremities, but rarely a general convulsion. The 
face is flushed ; the temperature subnormal at first ; pulse irregular ; 
respiration slow, irregular and of the Cheyne-Stokes type. The pupils 
are contracted; there may be convergent strabismus or conjugate devia- 
tion. Later a rise of temperature occurs, often reaching a high point ; 
swallowing is impossible ; dyspnoea, rapid pulse, cyanosis, due to paral- 
ysis of respiration, lead to death in from six to twenty hours after the 



448 THE EYE AND NERVOUS SYSTEM 

onset. In less violent cases paraplegia or a crossed paralysis manifests 
itself when the acnte stage is past, with exaggeration of reflexes. 

BULBO-PONTINE SOFTENING. 

The clinical differentiation between hemorrhage and softening is 
usually difficult and often impossible to make, as in the case of similar 
lesions in the cerebral hemispheres. The suddenness of onset, the 
presence of conditions in other organs predisposing to rupture of blood- 
vessels, notably hypertrophied heart with elevated blood-pressure, are 
suggestive of hemorrhage, though never absolutely conclusive. Soften- 
ing in the pons and oblongata is about three times as frequent as hemor- 
rhage, and occurs usually in syphilitic persons between the ages of thirty 
and fifty. Arteriosclerosis in some form is a necessary forerunner of 
softening, except in the rare cases of embolism. Men are affected as 
frequently as women. 

In general, the onset of severe symptoms is slower in softening from 
any cause than in hemorrhage, with the possible exception of embolism. 
Following general signs of arterial disorders, such as headache, vertigo, 
tinnitus, marked bulbo-pontine symptoms develop with greater or less 
rapidity. If the oblongata be particularly involved, through disturb- 
ance in the branches of the vertebral arteries, disorders of speech and 
difficulty in deglutition occur, with possible paralysis of the pharyngeal 
and laryngeal musculature. Contraction of the pupil, with smaller 
palpebral fissure, retraction of the globe and facial flushing, may occur 
from involvement of a sympathetic centre supposed to lie in the oblon- 
gata. Further disorders of sensibility and motility supervene if the 
lesion be extensive, due to injury of the pyramidal tracts, and interrup- 
tion of the sensory tracts lying deep in the oblongata. In unilateral 
lesions hemiplegia is a common accompaniment. There is also at times 
a dissociation of sensation of the usual character — loss of pain and tem- 
perature senses, with retention of the sense of touch, and ataxia. De- 
fective sense of position, astereognosis, and forced movements have also 
been observed. 

Involvement of the pons to the exclusion of the oblongata implies a 
freedom of the nuclei and peripheral distribution of the eighth to the 
twelfth nerves inclusive. The fifth, sixth, seventh, and often the third, 
show defects of various degree. Sensory disorder in the distribution 
of the fifth nerve is not uncommon. The pyramidal tracts, or often one 
tract, is involved, giving rise to hemiplegia, sparing the face if the 
facial nerve escapes injury both in its central and peripheral course. 
The so-called alternating or crossed hemiplegia is produced when the 



BULBAK AKD PSEUDOBULBAR DISEASES 449 

lesion lies in such a position as to involve the peripheral neurone of the 
seventh or sixth nerve, or if higher up, of the third nerve, and the 
pyramidal tract of the same side. The crossing of the pyramidal 
tract at a lower level leads to a paralysis of arm and leg on the opposite 
side, and of face or certain eye muscles on the same side as the lesion. 
Lesions involving the central path of the sixth nerve may also lead to 
conjugate deviation of the eyes towards or away from the lesion, de- 
pending upon whether the lesion be paralyzing or irritative. An injury 
to the common centre for the sixfh nerve on one side, and the third nerve 
(branch to the internal rectus of the opposite side), if it be destructive, 
will lead to a conjugate deviation away from the lesion. In the absence 
of knowledge regarding the extent of injury, definite rules concerning 
the diagnostic focal value of conjugate deviation cannot be laid down. 
Convergent strabismus is a result of implication of the sixth nerve, one 
or both. Nystagmus also is a common sign of pons lesion, to be ex- 
plained by disorder of the coordinative mechanism. Quadrigeminal 
lesions give rise to disorders in the distribution of the third nerve — 
external strabismus, ptosis, paralytic dilatation of the pupil — and of 
the less important fourth nerve. Symptoms of true bulbar paralysis, 
involvement of tongue, larynx, pharynx, may occur from interruption 
of the upper neurones of the hypoglossal, and vagoglossopharyngeal 
group in the pons. This effect is most markedly produced by a bilateral 
lesion. In such a lesion, the element of atrophy will naturally be lack- 
ing in the peripheral portion of the nerves involved. Injury of one 
fillet produces an imperfect sensory loss on the opposite side of the body, 
which may be associated with ataxia. Ataxia of the cerebellar type is 
probably produced by lesions of the superior peduncles and red nucleus. 
Pain is never a conspicuous symptom, which is in general true of intra- 
cerebral or spinal lesions. Uncontrolled emotionalism — forced laughter 
— is a frequent symptom in bulbo-pontine disease, and is perhaps best 
explained by the theory of partial or complete destruction of the paths 
of control, which must be closely associated if not identical with the 
upper neurones of the bulbar and pontine nerves. 

In general, the multiplicity of symptoms which may result from 
lesions of the oblongata or pons, or both, is apparent when one considers 
the complexity of anatomical structure in this portion of the brain. 
Considering, also, the fact that the ordinary lesions, producing acute 
bulbar paralysis, may vary widely in extent and destructiveness, it 
follows that the symptoms may range from slight and temporary dis- 
turbances in the functions of the bulb and pons up to conditions which 
are quickly fatal. It should, however, not be difficult to picture the 
29 



450 THE EYE AND NEKVOUS SYSTEM 

symptomatology of intermediate stages, if one bears in mind the func- 
tional significance of this lower portion of the brain stem. The follow- 
ing case illustrates sudden bulbar palsy of slight degree : 

A woman of fifty-five, of active life, well except for phlebitis some years ago 
and an unexplained attack of vertigo several months before, suddenly without pro- 
dromata, noticed distinct difficulty in speech, coming on without pain. This was 
associated with difficulty in swallowing, but without actual regurgitation. Exam- 
ination four days after the onset gave the following results: No definite arterio- 
sclerosis; heart sounds normal, except for accentuated aortic second. The eyes 
showed nothing abnormal, and sensibility was, in general, unimpaired. The upper 
cranial nerves were not involved. The tongue was protruded imperfectly, and all 
movements were possible, though weak and slow. Speech was thick, laborious, but 
understandable. There was paresis of the soft palate and of the pharynx. Swal- 
lowing was very difficult, but possible, when either liquid or solid was taken in 
small amount. The reflexes were, in general, active, but there was no evidence of 
hemiplegia or other paralysis of the extremities. Later reports showed rapid 
improvement. 

Such a case must be interpreted as due to a vascular bulbar 
disorder, not sufficiently extensive to lead to irremediable defect, 
It serves as a type of those bulbar affections which are slight in them- 
selves, but are indicative of the possibility of more serious results in the 
future. 

Course and Outcome. — As already suggested, if not immediately fatal, 
the tendency of this group of affections is towards improvement, usually 
leaving greater or less functional defect, except in the slightest cases, 
as for example, that quoted above. The reason for this is obvious from 
the character of the underlying pathological process, and is perfectly 
analogous to such processes elsewhere. Inasmuch as the conditions 
which lead to the onset of the symptoms are usually not materially 
influenced by treatment, the danger of subsequent attacks of similar 
character is evident. 

Treatment. — The treatment of acute bulbar paralysis should pri- 
marily be directed to the underlying cause, hemorrhage or softening, 
and should be chiefly preventive. If syphilis be thought a possible cause, 
and it should be suspected, especially in relatively young patients, an 
antisyphilitic cure should be administered vigorously. In other cases 
of arterial lesions, care should be taken to provide for adequate nutri- 
tion and to avoid the dangers of inhalation pnuemonia, as for the chronic 
progressive form. More important than the treatment of the actual 
condition are prophylactic measures, care of the kidneys and of the 
whole circulatory system. A strict dietary regime, avoidance of exer- 
tion and a restrained manner of life generally should be insisted upon 
in those cases which show the often vague signs of cerebral circulatory 





Figs. 5, 6.— Oblongata ; gumma involving region of bulbar nerves, fourth ventricle and a part of 
the cerebellum; various somewhat irregular bulbar and general symptoms ; basilar artery normal. 
(Photographs by L. S. Brown.) 



BULBAK AND PSEUDOBULBAR DISEASES 451 

disturbance, or which have undergone a slight apoplectic attack either of 
the brain or pons-oblongata region. Drugs are not serviceable, except to 
meet indications as they arise. 

BULBAR PARALYSIS FROM OTHER CAUSES. 

In addition to the foregoing somewhat definite type of bulbar 
disease, many other causes of disturbance in the function of this region 
have been described, some of which, on acount of their rarity and lack 
of relation to the general subject-matter of this book, may be cursorily 
considered. A few cases have been described (Ley den, Etter) of mye- 
litis of the bulb, with symptoms of acute bulbar paralysis. Leyden 
describes and pictures a case in which inflammatory changes and mul- 
tiple foci of hemorrhage were found in the region of the olives and else- 
where throughout the bulb, showing markedly on the floor of the ven- 
tricle. This condition is to be regarded as a local manifestation of the 
group of conditions usually described as acute encephalitis, or poli- 
encephalitis, and corresponds in a measure to the condition of superior 
encephalitis, of which special mention is hereafter made (page 452). It 
serves to demonstrate the fact, not infrequently observed, that inflam- 
matory processes, with a certain predilection for the gray matter, may 
occur anywhere throughout the central nervous system, the local symp- 
toms naturally depending upon the part involved. 

Trauma of the bulb occasionally occurs through stabbing wounds or 
fracture and dislocation of the upper vertebra?. Such injuries are 
usually fatal, but if not, the familiar symptoms are produced in pro- 
portion to the extent and severity of the lesions. 

Bulbar symptoms in the course of typhoid fever and leukaemia have 
been described (Eisenlohr) and also in multiple neuritis. An infective 
agent (streptococci) was found in the fatal typhoid fever cases, hemor- 
rhages and inflammatory changes occurred in the sheaths of bulbar 
nerves in the cases of leukaemia, and in rare cases it has been shown that 
a neuritis may affect the bulbar nerves. 

Tumors in the posterior cranial fossa, meningeal thickenings from 
syphilis or other cause, aneurismal dilatations or actual aneurisms of 
the basilar artery, and more rarely of the vertebrals (Oppenheim, 
Seimerling) may by compression lead to bulbar symptoms, often with 
involvement of the pyramidal tracts, and consequent spasticity of the 
extremities (Eigs. 5 and 6). The symptoms are naturally variable; 
they are usually slowly progressive, often with a final severe exacerba- 
tion. Owing to the tortuous course of the artery in compression by 
aneurism, the motor symptoms are irregular in distribution, and often 



452 THE EYE AND NERVOUS SYSTEM 

of the type of an alternating hemiplegia. The principles of diagnosis 
apply in general to cases of this type as to others, a peculiarity being 
gradual course, with final increase of symptoms, either through soften- 
ing or rupture of the aneurism. 

Cases of a family, congenital type of bulbar paralysis have been 
described by Remak, Londe, and others, to which allusion has previously 
been made, in which the upper branches of the facial nerves are apt to 
be involved, a fact possibly of diagnostic significance. 

SUPERIOR ENCEPHALITIS (WERNICKE). 

On a basis of three cases Wernicke, 12 in 1881, described a new symp- 
tom-complex, to which he gave the name of acute hemorrhagic superior 
poliencephalitis. A previous case, reported by Gayet in 1875, had not 
served to establish the identity of the process. Wernicke regarded the 
affection as an independent, acute inflammatory process in the region of 
the nuclei of the ocular nerves, fatal in from ten to fourteen days. The 
focal symptoms consist in associated paralysis of ocular muscles, of quick 
onset and rapid progress, finally leading to a practically total paral- 
ysis of the muscles involved, with the possible exception of the sphinc- 
ter iridis or the levator palpebrarum. The gait of the patient is ataxic, 
suggesting alcoholic intoxication. The mental state is markedly dis- 
turbed ; somnolence and agitation may both occur ; a mental condition 
strongly suggesting dilirium tremens may form part of the clinical 
picture. In the three cases described by Wernicke changes of an in- 
flammatory character were observed in the optic disk. Alcohol played 
a prominent part in the etiology of the affection in two cases, and sul- 
phuric acid poisoning in the other. The essential pathological altera- 
tion in each case was multiple punctiform hemorrhages in the dorsal 
portion of the third ventricle and the region of the aqueduct of Sylvius, 
including especially the nuclei of the ocular nerves. 

Subsequent observations have extended the knowledge of the acute 
poliencephalitis described by Wernicke. Influenza is probably an im- 
portant factor in etiology as well as other infectious diseases, and 
poisonings of varied character. The prognosis is also not so invariably 
bad as at first supposed, nor may the pathological anatomy be regarded 
as uniform. 

In twenty well observed cases of Wernicke's hemorrhagic polien- 
cephalitis, fifteen with autopsies, Wilbrand and Saenger 13 note the fol- 



12 Wernicke: Lehrbuch der Gehirnkrankheiten, 1881, §47, p. 229. 

13 Wilbrand and Saenger : loc. cit., p. 270. 



BULBAK AND PSEUDOBULBAR DISEASES 453 

lowing facts of importance with reference to the ocular conditions : In 
nine cases there was ptosis of varying degree ; in all the cases, excepting 
two, in which no observation on the condition of the muscles is made, 
the ocular muscles were variously involved, affecting both the third and 
the sixth nerves, giving rise to more or less immobility of the eyes, with 
accompanying diplegia, nystagmus, and strabismus ; the pupils showed 
varying reactions to light, from normal to complete immobility; prac- 
tically no observations were made on the power of accommodation, for 
obvious reasons, in view of the constant palsy of the external muscles ; 
ophthalmoscopic examination showed changes in a few instances, of the 
character of hyperemia, unusual pallor of parts of the disk, and occa- 
sionally papillitis. Various nuclear and peripheral degenerations were 
found post-mortem. In general, the ocular conditions showed no ten- 
dency to conform to any one type. 

The position definitely taken by Wernicke that superior polien- 
cephalitis is a wholly analogous affection to poliomyelitis and other in- 
flammatory affections throughout the brain stem has been amply borne 
out by subsequent observations. This is shown, first, by the essential 
identity of the pathological findings in these variously localized pro- 
cesses, and secondly, by the many transitional forms which have been 
described. 14 Encephalitis, on the one hand, superior poliencephalitis, 
inferior poliencephalitis, giving the symptoms of an acute bulbar paraly- 
sis, poliomyelitis, and finally poliencephalomyelitis, no doubt represent 
simply different expressions of a common underlying cause, the symp- 
toms naturally varying widely with the portion of the nervous system 
involved. 

The analogy may usefully be carried further in considering the 
more chronic forms of degeneration of the upper cranial nerves, chronic 
ophthalmoplegia and allied conditions, as being similar in character 
to chronic progressive bulbar paralysis, already described (page 
436), and to the spinal form of progressive muscular atrophy. The 
transition between these lesions and the degree of acuteness of onset 
together make possible most varied symptomatic manifestations, of 
which a certain number stand out conspicuously, and have, therefore, 
been regarded as clinical entities. Among these chronic ophthalmo- 
plegia is important. 

CHRONIC OPHTHALMOPLEGIA. 

In the great majority of cases chronic, usually progressive, ophthal- 
moplegia, forms a part of a more general process, as for example, tabes 

"Taylor: Poliencephalitis and Allied Conditions, Boston Med. and Surg. Jour., 
cxlviii. 634, 1903. 



454 THE EYE AND NERVOUS SYSTEM 

or dementia paralytica, and is frequently associated with progressive 
bulbo-pontine degenerations of motor nuclei, and at times with spinal 
cord lesions of similar character. As an early manifestation of one of 
these more extensive affections, as in those cases in which it dominates 
the clinical picture, chronic ophthalmoplegia is deserving of special 
consideration. 

The symptom-complex was first described by von Graefe. Its de- 
velopment is slow and its course long, in contrast to progressive bulbar 
paralysis, due to the fact that the process is located in a region of the 
brain not essential to life. Although varying widely in detail, the 
disease begins usually with diplegia or ptosis, followed by a very gradual 
extension of the palsy to the external ocular muscles of both eyes ; the 
accommodative mechanism and the levator palpebrae superioris are 
often spared. Later the internal muscles may be involved, or in other 
cases they may be affected from the beginning. The progressive in- 
volvement of the muscles points always toward nuclear degeneration as 
a cause, easily explained by the complicated anatomical arrangement of 
the nucleus of the third nerve. As the process continues, the eyeball 
may ultimately become wholly immovable, and the levator palpebrae 
and orbicularis oculi also be involved. In rare cases the disease is 
checked when the ocular paralysis is complete (Striimpell). It is, how- 
ever, usual for the degeneration to extend to other nuclei, with an ulti- 
mately fatal outcome. 

Pathological Anatomy. — The usual lesion is a progressive degenera- 
tion of the cells of the nuclei of the third, fourth and sixth nerves, pre- 
cisely analogous to that described under progressive bulbar paralysis. 

PSEUDOBULBAR PARALYSIS. 

Knowledge of this type of bulbar paralysis dates from 1872, when 
Joffroy first called attention to the fact that the symptoms of bulbar 
lesion may occur without disease of the oblongata, the essential altera- 
tions being in the cerebral hemispheres. An almost simultaneous pub- 
lication by Jolly of a case with bulbar symptoms from cerebral multiple 
sclerosis, and in 1877 a report of cases by Lepine and Barlow estab- 
lished the essential identity of the symptom-complex. Lepine first used 
the term " pseudobulbar" as applied to these cases. Since that time 
papers have appeared by Kirchoff, Fere, Jolly, Oppenheim, and Siemer- 
ling, Russell and Taylor, Becker, Remak, Colman, Oppenheim, 
Bouchard, Karplus, Weisenburg, and others. From these various ob- 
servations it is apparent that a bilateral destructive lesion of the cortico- 
bulbar tracts is sufficient to produce the general symptoms of bulbar 




. fig. 7.— Poliencephalitis superior and inferior. The symptoms of gradual onset were dysphagia, 
paresis of soft palate, respiratory and cardiac disturbances, vertigo, somewhat exaggerated tendon 
reflexes (but no weakness of limbs), bilateral ptosis, intense external and internal ophthalmoplegia- 
diplopia, and pallor of optic disks. (Unreported case seen by Dr. Spiller with Dr. Thorington.) 




Fig. 8.— Same patient as Fig. 7. Photograph shows the bilateral ptosis and the inability to look upward. 







Figs. 9, 10.— Intense muscular atrophy occurring in encephalo myelitis. (Case of Dr. Spiller.) 




Fig. 11. — A case of myasthenia gravis, with paralysis confined to the ocular muscles. (Reported by 
Drs. Spiller and Buckman, Amer. Jour, of the Med. Sciences, April, 1905). The ptosis of the right eye 
is not so great as that of the left. The photograph was taken one or two minutes after glasses, one of 
which was opaque, were removed. At times the patient was able to open both eyes fully. 




Fig. 12.— Same patient as shown in Fig. 11. The eyeballs are almost completely covered. The photo- 
graph was taken after the glasses had been removed three or four minutes. 



BULBAE AND PSEUDOBULBAE DISEASES 455 

palsy, presumably by a cutting off of the upper paths of voluntary 
control. 

Pathological Anatomy. — The usual post-mortem findings are arterio- 
sclerotic changes, with the resultant areas of softening and degeneration. 
The lesions are usually bilateral, though a few cases of bulbar palsy 
have been observed, in which the cerebral lesions were confined to one 
hemisphere. The usual locations of the softened areas are the cerebral 
white matter, internal and external capsules, central ganglia, particu- 
larly in the lenticular nuclei, and more rarely in the cortex. That such 
lesions are sufficient to produce the symptoms of bulbar paralysis has 
been shown by adequately studied cases. Oppenheim and Siemerling, 15 
however, warn against the too hasty acceptance of cerebral lesions alone 
as explanatory of the symptoms. In the investigation of five cases, in 
which the pons and oblongata were studied serially, it was found that 
multiple small areas of softening were present in such a position as to 
require consideration in the production of symptoms. They very rightly 
maintain, therefore, that the cerebral origin of the symptoms should not 
be insisted upon until a careful microscopic examination of the pons- 
oblongata region is made. This apparently was not done in many of the 
earlier cases reported. 

It seems advisable, therefore, to divide the cases on the pathological 
side into cerebral, and cerebrobulbar, recognizing the fact that a purely 
cerebral form is less usual than at first supposed. The cerebrobulbar 
forms evidently may not differ widely in their pathological anatomy 
from certain forms of acute bulbar paralysis, due to focal softening in 
the pons or oblongata, a general arteriosclerosis being the underlying 
condition in both cases. Oppenheim and Siemerling have also called 
attention to the importance of pressure exerted on the oblongata by a 
dilated, aneurismal or arteriosclerotic vertebral or basilar artery, in pro- 
ducing symptoms which might otherwise be attributed to more distant 
lesions. Eecognizing these various possibilities, the following lesions 
are types of those which have been described as causative of the symp- 
toms : bilateral softening in lenticular nucleus, parietal lobe, cortex of 
island of Eeil, tempero-sphenoidal lobe (Fere) ; lesions in both cap- 
sules (Colman) ; cortical cystic tumors (Picot and Hobbs) ; cortical 
changes in an epileptic (Bouchard) ; diffuse lesions of hemispheres 
(Becker) ; microgyria (Oppenheim). 

Symptomatology. — Considering the rather wide-spread lesions in the 
brain which are required to produce disturbance in the bulbar innerva- 

15 Oppenheim and Siemerling: Versammlung Deuts. Naturforscher, Berlin. 1886. 



456 THE EYE AND NERVOUS SYSTEM 

tion, it follows that the symptoms are less sharply confined to this region 
than in processes before discussed. Various paralyses of the extremi- 
ties, hemiplegia, paraplegia, together with aphasia, hemianopsia, and 
general mental defect, may form a part of the clinical picture, together 
with distinctly bulbar symptoms. 

The development of the affection is characterized by repeated ex- 
acerbations, each leaving the patient with a further disability, and in- 
creased mental enfeeblement. Characteristic, and found only in this 
type, is progressive dementia, with various supervening conditions of 
confusion and excitement, forming part of the picture of a wide-spr A ead 
arteriosclerosis. The paralyses of extremities are naturally dependent 
upon the position of lesions interrupting motor conduction. The sig- 
nificant symptoms for present consideration are those dependent upon 
lesion of the tracts to the bulbar nerves, whence comes the simulation of 
actual lesion of the oblongata. Loss of the power of speech, difficulty 
in deglutition, paresis of lower branches of the facial nerve (lip mus- 
cles) and of the palate, occur in a form superficially indistinguishable 
from true bulbar paralysis. The muscles involved are, however, not 
atrophied (disease of upper motor neurone) and the electrical reactions 
are not affected. Other symptoms of less constant occurrence are: 
Weakness of muscles of mastication; increased pulse-rate and aryth- 
mia ; optic atrophy or neuritis ; occasional impairment of bladder and 
rectal function ; rarely anaesthesia in various localities. More frequent 
are respiratory disorders: attacks of dyspnoea and Cheyne-Stokes 
breathing. As in other forms of bulbar paralysis, uncontrolled 
expression of the emotions is frequent; due, no doubt, to a diminu- 
tion of inhibition through destruction of paths of control, especially, 
it has been suggested, those from the cortex to the optic thalamus. 
Certain observations seem to show that a bilateral break in tracts 
from the cortex to the nuclei of ocular nerves may lead to disorder 
in movement of the eyes, of such a character that voluntary control 
is difficult, whereas they may still follow an object (Oppenheim and 
others). 

The symptoms usually first manifest themselves after an apoplectic 
attack, often slight in itself, but succeeded at varying intervals by 
others, until finally the clinical picture is fully developed. Much more 
rarely the disease progresses slowly after an acute onset. The outcome 
is dependent upon the position, character and extent of the arterio- 
sclerotic process. 

It is evident in this, as in other inflammatory and degenerative pro- 
cesses, which have been considered in the foregoing pages, that an abso- 



BULBAE AND PSEUDOBULBAR DISEASES 457 

lutely uncomplicated clinical type is rarely found. The distinction be- 
tween cerebral and cerebrobulbar forms is often impossible to make 
clinically, and again the relationship between these forms and acute 
softening of the bulb is often confused. Many unclassified transitional 
forms of bulbar disease, dependent upon general arteriosclerosis, must 
be recognized, which it is not necessary to describe in detail, provided the 
underlying principle of the genesis of the symptoms be understood. 

Prognosis and Treatment. — The outcome of pseudobulbar paralysis is 
ultimately fatal, through the increasingly disastrous results of the un- 
checked arteriosclerotic process. Remissions' are frequent, but the gen- 
eral tendency is progressive, and little can be done by way of treatment, 
beyond a careful regulation of the patient's life in all particulars, and 
attention to the details of feeding and prophylaxis, as stated in a 
previous section. 

MYASTHENIA GRAVIS. 

This condition is also described under the following titles: Erb's 
disease; asthenic bulbar paralysis (Strumpell) ; asthenic paralysis; 
myasthenia gravis pseudo-paralytica (Jolly) ; bulbar paralysis without 
discoverable anatomical lesion ; Erb-Goldflam or Hoppe-Goldnam symp- 
tom-complex. 

Although an undoubted case was earlier described by Wilks, the 
credit of first calling definite attention to this affection belongs to Erb, 
who, in 1879, 16 on a basis of three cases described the group of symp- 
toms, since known as myasthenia gravis. He drew special attention to 
the symptoms of ptosis, weakness of the muscles of mastication and of 
the neck muscles. No autopsy was obtainable in his one case which 
died ; but a bulbar involvement was naturally suspected, from the char- 
acter of the objective signs. Less importance was attached to coincident 
weakness of the tongue, muscles of deglutition and extremities. Mus- 
cular atrophy suggested a chronic poliencephalitis. In 1886 Jendras- 
sik 1T published a probable case of the same affection, and in 1887 
Oppenheim 18 called attention to a similar, if not identical, symptom- 
complex, which he then regarded as unique, apparently not considering 
Erb's earlier cases as coming into the same category. In Oppenheim's 
case a painstaking post-mortem examination showed no lesions, and the 
affection was, therefore, characterized as a chronic progressive neurosis 

16 Erb: Arch. f. Psych., ix. 336, 1879. 

17 Jendrassik : Arch. f. Psych., xvii. 301, 1886. 
^Oppenheim: Virchovv's Archiv, cviii. 522, 1887. 



458 THE EYE AND NERVOUS SYSTEM 

manifesting itself essentially by the symptoms of a glossopharyngo- 
labial paralysis without atrophy. Goldflam, 19 in 1891, more clearly de- 
fined the condition by laying stress upon the element of muscular fatigue 
as distinguished from actual paralysis, a matter later amplified by 
Jolly 20 in regard to electrical alterations, which he defined as the " my- 
asthenic reaction." These changes consist essentially in a rapid tiring 
of the muscles under strong faradic stimulation, manifested by an 
increasing diminution in contractility. 

An important contribution to the general subject was made by 
Hoppe 21 in 1892, from Oppenheim's laboratory, in which he described 
a case of general motor weakness, but especially of the cranial nerves, 
of slow progression, in some regions going on to paralysis, with electrical 
alterations, but without atrophy; final fatal outcome and absolutely 
negative post-mortem findings. Hoppe considers this case not identical 
with Erb's, chiefly on the ground of complete absence of muscular 
atrophy. He further questions the utility of the term neurosis, as ap- 
plied to the condition by Oppenheim, and regards it as probably of 
cortical origin. 

Since this preliminary work, which established the disease on a 
fairly firm basis, a very large number of cases have been reported, with 
several critical reviews of the subject and ample bibliographical lists. 22 
The general conception of the affection has not been materially altered 
by the work of the past few years, although a certain light has been 
thrown upon its possible pathological anatomy by Weigert, 23 and very 
recently by Hun and his collaborators, Drs. George Blumer and G. L. 
Streeter, and by Drs. C. W. Burr and B. F. Stahl. 

Pathological Anatomy. — According to Hun's tabulation, autopsies in 
thirty-two cases have shown negative findings in the nervous system in 
seventeen cases. In the remaining cases lesions found in the nervous 
system were slight recent hemorrhages, and uncertain changes in motor 
nerves and nuclei of the oblongata, together with certain abnormalities 
of development, which cannot be brought into causal relationship with 
the clinical course of the disease. Findings outside the nervous system 
are probably of more significance; in fact, there is small evidence to 

19 Goldflam: Neurolog. Ctb., x. 162, 1891; also Deuts. Zeitsch. f. Nervenheilk., 
iv. 312, 1893. 

20 Jolly: Berl. klin. Woch., xxxii. 1, 1895. 

21 Hoppe: Berl. klin. Woch., xxix. 332, 1892. 

22 The more important of these are Campbell and Bramwell : Brain, xxiii. 277, 
1900; Myers: Jour, of Pathol, and Bacteriol., viii. 306, 1903; Hun: Albany 
Medical Annals, xxv, 28, 1904 (124 references). 

23 Weigert: Neurolog. Ctb., xx. 597, 1901. 



BITLBAK AND PSEUDOBULBAR DISEASES 459 

show that a primary affection of the nervous system is in any way 
responsible for the symptoms supposed to be characteristic of the affec- 
tion. Weigert's observation, made in 1901, is possibly of importance 
in the light of certain other similar findings, and especially of Hun's, 
to which allusion has been made. In a case of myasthenia gravis Wei- 
.gert, in a routine post-mortem examination, found an extensive infiltra- 
tion of the muscles with lymphoid cells, which he regarded as metastases 
•of a malignant tumor of the thymus, of lympho-sarcomatous character. 
The appearances were so striking in this case that Weigert was inclined 
to think that had similar changes been present in all previous cases, they 
•could not have been overlooked, and, therefore, that one is not justified 
in assuming that such a lesion is the constant cause of myasthenia. 
Hun has recently described a case identical with Weigert's, and on the 
I)asis of this fact, taken in conjunction with observations of similar 
•character made by Link, Oppenheim and Goldflam, strongly advocates 
the theory that the secret of the pathological anatomy probably lies in 
these or closely analogous alterations. A very similar condition has 
recently been reported by Burr and Stahl before the Philadelphia Neu- 
rological Society, and Burr still further discusses the subject in the 
Journal of Nervous and Mental Disease for March, 1905. The final 
-explanation of the muscular weakness and myasthenic reaction Hun 
seeks in a disturbance of the function of the muscle end-plates, brought 
about by interference with the lymph circulation in the muscles. To 
^explain the cause of the infiltration of the muscles with lymphoid cells 
•carries one wholly into the field of speculation and requires no discus- 
sion here. 

Symptomatology. — The disease is ordinarily insidious in onset, and 
characterized essentially by weakness and rapid tiring of muscles. In 
uearly one-half the reported cases, according to Hun, 24 ptosis is a very 
nearly symptom. In forty-five per cent, of the cases ocular palsy, either 
ptosis, or diplegia, occurred as the earliest symptom. In a smaller pro- 
portion, twenty-three per cent., involvement of the facial, hypoglossal 
and accessory nerves was the first symptom. A somewhat larger number 
of cases gave as the earliest sign of the disease weakness of the arms or 
legs or both. As the disease develops the usual characteristic picture is 
double ptosis, double facial paresis, paralysis of the bulbar nerves, asso- 
ciated with dyspnoea, and general bodily weakness. Among these symp- 
toms the ocular palsies occupy probably the most conspicuous place, and 



24 Hun's paper, loc. cit., contains a most valuable analysis of earlier cases in 
their various aspects. 



460 THE EYE AND NEKVOUS SYSTEM 

of individual symptoms ptosis is the most frequent, occurring sooner or 
later in upward of eighty-five per cent, of all cases. Various external 
muscles of the eye are also frequently involved, but the internal muscles 
governing the pupil are very rarely included in the muscular weakness. 

Wilbrand and Saenger 25 in an analysis of forty-three cases find 
diplegia in fourteen; ophthalmoplegia exterior in nine; weakness of 
the sphincter iridis and ciliary muscles in one; unilateral ptosis in 
three; bilateral ptosis in twenty-nine; disturbance in the distribution 
of the facial nerve, a number involving the orbicularis oculi, in twenty- 
five. Gowers 26 has recently called special attention to the affection of 
the eye muscles in connection with a study of three personal cases. At 
first sight the ophthalmoplegia simulates the variety due to nuclear de- 
generation, but the differences are probably significant. In the myas- 
thenic form, the muscles moving the eyeball downward are less involved 
than those moving it upward, the lateral muscles are constantly but 
irregularly involved, but differing in degree in associated muscles and 
also showing striking variations at different times. There is no corre- 
spondence in some of the cases between the degree of ptosis and the im- 
pairment of upward movements of the eyes. The orbicularis palpe- 
brarum is often included in the weakness, and the frontalis may be. 
Gowers also draws attention to a characteristic facial expression, which 
he calls the " nasal smile," brought about by a failure of the normal 
movement of the corners of the mouth, the furrow caused by the attempt 
to smile being wholly confined to the upper lip, and ceasing towards the 
corners of the mouth. The cause of this peculiarity is a weakness of the 
levator labii superioris. 

The involvement of muscles supplied by the facial nerve, in general, 
is a frequent sign, giving rise to so-called facial diplegia. The muscles 
of mastication, speech, deglutition and phonation may all be weak, and 
these disabilities, if conjoined with weakness of the neck, body, and 
extremity muscles, reduce the patient to a condition of practical help- 
lessness. The face is expressionless, the eyes partially closed, the jaws 
are moved weakly, speech is impossible, swallowing is difficult, the head 
cannot be voluntarily supported, dyspnoea may come on, and the skeletal 
muscles be too weak to hold the body erect. 

In spite of such a wide distribution of symptoms, the condition is 
in every instance due to a rapid tiring of the muscles, associated as the 

25 Wilbrand and Saenger: Neurologie des Auges, 1899. 

26 Gowers: Clinical Lectures, Blakiston, 1904, Lecture on Myasthenia and Oph- 
thalmoplegia, p. 221. 



BULBAK AND PSEUDOBULBAR DISEASES 461 

process develops with more or less persistent weakness. As a rule those 
muscles which are most constantly in use show the greatest weakness as a 
result of tiring, but this is by no means invariably the case. The 
order of involvement of the muscles is also not always as given above, 
beginning in the eyes, and extending gradually downward. The reverse 
may occur, and not infrequently the distribution of the weakness is 
wholly irregular. 

The association of weakness and abnormally rapid tiring of muscles 
on use must be regarded as an essential symptom of the disease. To 
this must be added the peculiar action of the affected muscles on elec- 
trical stimulation, first carefully studied by Jolly. This so-called " my- 
asthenic reaction" consists in a rapid diminution in contractility when 
stimulated by a tetanizing faradic current. The galvanic current has a 
much less tiring effect according to most observers, although opinions on 
this point are not altogether uniform. Reaction of degeneration does 
not occur, and muscular atrophy forms no part of the clinical picture. 
The relation between voluntary and electrical (faradic) contraction of 
the muscles remains undetermined. Jolly found that exhaustion took 
place when the muscles were voluntarily contracted, in the same degree 
as when electrically stimulated ; others have had a different experience. 
The myasthenic reaction, though not constant, occurs in some form in 
a large majority of the cases. In fatigue brought about by other causes, 
and in certain cerebral and spinal diseases, an electrical reaction similar 
to that occurring in myasthenia has been described, but the reaction, 
nevertheless, remains a characteristic and striking feature of the disease. 

The sensibility is not disturbed in the affection, and the mental 
faculties remain unimpaired. The knee-jerks at times show a tendency 
to exhaustion; the sphincters are not involved. Naturally a great 
variety in the extent, grouping and degree of the muscular exhaustion 
leads to much diversity in the clinical picture. In general, the combina- 
tion of cranial nerve weakness, and especially ptosis with accompanying 
weakness of the skeletal muscles ; absence of muscular atrophy, with 
unaltered electrical reaction, apart from rapid tiring on faradic stimula- 
tion ; tendency to variations from time to time ; unimpaired sensibility 
and freedom from mental defect, form a symptom-complex which is 
found in no other condition. 

Etiology and Course. — The disease ordinarily occurs in young adults, 
without reference to sex or hereditary influences. There is no uni- 
formity in the various conditions which have been described antecedent 
to the actual appearance of the disease. Even if the finding of a 
lymphoid infiltration of the muscles with disordered lymphatic circula- 
tion be regarded as etiologically significant, the ultimate cause still 



462 THE EYE AND NEKVOUS SYSTEM 

remains obscure. To say that the affection is due to a toxaemia is rather 
to restate the question than offer a solution. 

The onset of the disease is usually insidious, and its course slow, 
often with marked remissions followed by exacerbations. Successive 
groups of muscles are usually involved, often irregularly. Temporary 
improvement may take place for months or even years, with final relapse 
to the former or to a worse condition. Variability in the development 
of symptoms is so constant a feature of the disease as to be of great 
importance in the diagnosis. In ninety-eight of the tabulated cases the 
intensity of the symptoms varied from day to day and in sixteen only 
was the progression steady. A few cases have been described in which 
the onset was acute, and the disease fully developed in the course of a 
few weeks; these are, however, wholly exceptional. Death has ter- 
minated the affection in something less than fifty per cent, of reported 
cases; a small number, seven, are regarded as cured, and the others, 
fifty-seven out of one hundred and fourteen, are still living. 

Treatment. — The essential of treatment is the avoidance of muscular 
fatigue. In serious cases the patient should be kept in bed, and warned 
against exertion of any sort. Electricity should be applied with great 
care. Central galvanization is advisable. Oppenheim quotes a case 
in which a fatal outcome followed an attempt at artificial feeding. 
The food should be of such a character that the effort of swallowing may 
be reduced to a minimum ; the possibility of sudden death from suffoca- 
tion should be borne in mind. In milder cases less care is required, but 
the results hitherto attained are not of such a character as to warrant the 
hope that the course of the disease can be essentially modified. Appro- 
priate tonic and general hygienic and symptomatic treatment will ac- 
complish something toward the amelioration of the general condition. 

PARALYZING VERTIGO (GERLIER'S DISEASE). 

The disorder known as Paralyzing Vertigo, or Gerlier's Disease, was 
first described by a Swiss physician, Gerlier, 27 in 1887. Since that time 
he has contributed a series of articles, amplifying his first detailed 
description especially on the clinical side. David, 28 Haltenhoff, 29 
Eperon, 30 Ladame, 31 and Sulzer 32 have made communications of less 



27 Gerlier: Rev. Med. de la Suisse Romande, vii. 1, 260, 1887; idem, viii. 22, 86, 
1888; idem, xi. 201, 260, 1891. 

28 David: Rev. Med. de la Suisse Romande, vii. 65, 1887. 

29 Haltenhoff: Prog. Med., xv. 515, 1887. 

30 Eperon: Rev. Med. de la Suisse Romande, ix. 38, 1889. 

31 Ladame: Critical Digest. Brain, xii. 534, 1890. 

32 Sulzer: Rev. Med. de la Suisse Romande, xiii. 695, 1893. 



BULBAK AND PSEUDOBULBAK DISEASES 463 

importance on the clinical aspect of the subject or in criticism of Ger- 
lier's views. 

The affection has been observed in Europe only in Collex, a canton 
of Geneva, and in seven surrounding hamlets. Its victims are workers 
in the fields, and particularly cowherds. Vigorous men in the early 
period of life are attacked, during the summer months only, and those 
exposed to the worst hygienic conditions are usually the first and most 
seriously afflicted. 

Symptomatology. — Three symptoms are characteristic; pain in the 
region of the neck, muscular weakness and ocular disorders. These 
may be variously combined, and occur with varying degrees of intensity 
in the same person at different times or in different persons. Vertigo, 
as ordinarily understood, is a less conspicuous symptom. 

The neck pain is described as a sense of constriction, with a ten- 
dency to torticollis, the pain often radiating to the back. The muscular 
weakness is confined to those muscles normally under control of the 
will, and the extensors are more commonly affected than the flexors. 
The neck muscles are usually involved, but unsymmetrically on the two 
sides. The disturbance of the eyes is characterized by ptosis, usually 
unequal in the two eyes and of varying degree. This is not accompanied 
by strabismus, or palsy either of the external or internal ocular muscles, 
and there is at times a diminution in the visual fields. The fundus has 
apparently not been extensively examined, but so far as they have gone 
the ophthalmoscopic findings are negative, with the exception of an 
observation of Eperon, in which he found papillary hyperemia and 
retinal hemorrhage, and a similar finding by Sulzer. A subjective sen- 
sation of clouding of vision, preceding the onset of ptosis, accompanied 
by diplegia, and vertigo, rarely circular in character, often occurs. 

A typical attack may be described as follows : The patient, pre- 
viously well, is suddenly attacked by pain in the neck and back; his 
sight is clouded to the point of temporary blindness; ptosis develops 
rapidly along with general weakness of neck and body muscles ; stand- 
ing erect becomes difficult ; he reels and has all the appearance of drunk- 
enness. After a period not exceeding ten minutes, complete recovery 
takes place. During an attack the tendon reflexes are preserved, at 
times increased; the skin sensibility is intact, false trismus occurs, 
swallowing is often impossible, and general motor weakness dominates 
the clinical picture. The head often falls forward on the chest, due to 
weakness of the neck muscles, which, with the ptosis cutting off vision, 
renders the patient for the time being wholly helpless. Nausea does not 
occur. Many of the attacks are very slight and merely momentarily in- 



464 THE EYE AKD KEKVOUS SYSTEM 

capacitate the patient, but the foregoing symptoms invariably are de- 
veloped in some degree. 

Etiology. — Nothing definite is known of the cause of the attacks. 
Gerlier was of the opinion, though it was not experimentally verified, 
that inasmuch as peasants working and sleeping in stables were usually 
affected, the cause was to be sought in an infection or " miasm," only 
active in the warm months of summer, derived from the soil of the 
stables. The malady has been shown to be seasonal, always checked by 
the onset of cold weather ; the attacks, furthermore, occur usually with 
striking regularity in the afternoon, ceasing about an hour after sunset. 
They are, however, unaffected by the administration of quinine. Attacks 
are induced by excitement, brilliant light, by gazing fixedly at an object, 
or by the contemplation of a wide space. Ladame regards the affection 
as hysterical, and wholly due to suggestion. This Gerlier rather in- 
dignantly denies, giving as a conclusive argument the fact that he has 
observed the same attacks in cats, which presumably are not suggestible. 
Eperon is inclined to attribute the attacks to a meningeal hyperemia, 
possibly allied to sunstroke ; Sulzer considers the possibility of lesions of 
the occipital lobes, accompanied by descending neuritis of the optic 
nerves. 

Miura in 1897 described a similar affection occurring in the north- 
ern provinces of Japan, to which he gave the name " Kubisagari." The 
clouding of vision he regarded as a nervous asthenopia, and the diplegia 
present in his cases as an insufficiency or paresis of the internal recti. 
The possible relationship of these conditions with asthenic bulbar paral- 
ysis, and also with the rare family periodic paralysis, should be borne 
in mind. 

Treatment. — Iodide of potash, cold bathing, and generally improved 
hygiene have been recommended. The disease is never fatal, and is 
invariably terminated by the onset of cold weather. 



CHAPTER XL 

ARTEEIOSCLEEOSIS ; MULTIPLE SCLEEOSIS ; PSEUDOSCLE- 
EOSIS; DIFFUSE SCLEEOSIS; FEIEDEEICH'S DISEASE; 
PAEALYSIS AGITANS. 

By WILIAM HIRSCH, M.D. 

ARTERIOSCLEROSIS. 

AcfcoRDiiTO to our present views, arteriosclerosis forms merely a 
part of a general angiosclerosis, — i.e., an affection of the entire vascular 
system, which may be due either to a general regressive metamorpho- 
sis, to the influence of toxic agents, or to congenital abnormalities in the 
structure of the vessels. Inasmuch as the integrity of every organ 
depends on its proper nutrition and blood supply, and this again is 
dependent on an unimpaired vascular system, it is evident that arterio- 
sclerosis is apt to produce morbid changes in any and every part of 
the body. 

During the earlier stages of arteriosclerosis the affections of the 
various organs consist merely of functional disturbances, such as irreg- 
ularities in circulation are apt to produce. But as soon as the morbid 
changes in the blood-vessels become more marked, and consequently the 
circulatory disturbances more severe, the various organs are apt to 
become subject to organic diseases. 

The functional disturbances which a general arteriosclerosis is apt 
to produce in the eye correspond to analogous symptoms in other organs. 
The muscles are easily fatigued .and are lacking in endurance and 
power of resistance. Patients complain of difficulty in reading or of 
headaches after using the eyes, in spite of normal or by glasses cor- 
rected lenses. Besides these disturbances in accommodation and con- 
vergence, the diminution of muscular tonus may manifest itself by 
slight difficulties in moving the eyeballs, by incomplete and transitory 
ptosis, or by slight nystagmus, especially in horizontal movements of 
the eyes. 

Arteries which are undergoing sclerotic changes are subject to such 
functional disturbances as spasm and temporary paralysis. The well- 
known symptom of a temporary scintillating scotoma is in all proba- 
bility produced by a spasm of the blood-vessels supplying the cortical 
lobe. Such spasms may occur also in the blood-vessels of the retina, 

465 
30 



466 THE EYE AND NEKVOUS SYSTEM 

and then produce specks before the eyes, scintillation, and peculiar 
color-sensations. Another symptom which is evidently produced by 
vascular spasm, and therefore may occur in arteriosclerosis, is that of 
so-called " paroxysmal" or " epileptiform" amaurosis. The symptom 
consists of a sudden attack of blindness in one or both eyes, lasting from 
a few seconds to several hours, without any ophthalmoscopic changes. 

In some cases of general arteriosclerosis (according to some writers 
in 50 per cent.) a sclerotic process of the retinal arteries can be recog- 
nized with the ophthalmoscope. While under normal conditions the 
wall of a retinal vessel is invisible, a sclerotic artery may show a gray- 
ish shadow along its course. At some places the lumen of the vessel may 
have become narrower on account of the thickening of the wall. Such 
places appear in the ophthalmoscopic picture as fusiform yellowish- 
gray patches. In extreme cases the well-known red lines on the retina 
seem to be changed into thin white threads. 

Another objective symptom of sclerosis of the retinal vessels may be 
the presence of an arterial pulse, which, of course, must be distinguished 
from the pulse of pressure or the intermittent entrance of blood. Ac- 
cording to Thoma, this phenomenon occurs during the early stage of 
arteriosclerosis, and differs from the pulsation in aortic insufficiency by 
its lateral motions and flexion of the vessels, so that it is a locomotion 
rather than a true change of calibre. Thoma explains this phenomenon 
by the fact that in the beginning of arteriosclerosis the walls of the ves- 
sels are more elastic than under normal conditions; as soon, however, 
as the thicker layer of connective tissue in the intima between endothe- 
lium and elastica interna has been formed, the wall becomes firmer again 
and gradually loses its elasticity. 

Sometimes the shape of the pupil may become slightly changed. 
It may be oval instead of circular or show some other small irregulari- 
ties of its circumference. 

Occasionally an ccdematous swelling of the lids may occur or slight 
hemorrhages may take place in the conjunctiva or retina. These hem- 
orrhages into the substance of the eye, although mostly harmless in 
themselves, may be of great prognostic value, inasmuch as similar proc- 
esses in any other part of the body would necessarily escape observa- 
tion. Hasket Derby found that of 31 patients who consulted him 
for retinal hemorrhages nearly 50 per cent, died within two years, 
either from heart disease or from apoplexy. 

In cases of cerebral arteriosclerosis the optic nerve may be 
injured by direct pressure of an atheromatous vessel. The anatomical 
conditions for such an occurrence are especially favorable in the optic 



ARTERIOSCLEROSIS 467 

canal. Even under normal conditions the external shape of the optic 
nerve may undergo various changes. By its lower surface the nerve 
rests on the internal carotid artery, which, of course, performs most 
vigorous pulsations. The ophthalmic artery, which branches off in the 
form of an upwardly convex arch, also pulsates very strongly, espe- 
cially at its origin. Every pulsation of these two vessels presses the 
nerve against the fibrous roof of the intracranial part of the optic canal, 
and thus exerts an important influence on the shape of the nerve in this 
region. It is evident that this influence must be greatly increased if 
the diameter of these vessels becomes enlarged, their walls hardened, 
and their course more curved by a sclerotic process. 

The pressure which a sclerotic ophthalmic artery is apt to exert on 
the optic nerve may show itself in various ways. As a rule, the artery 
produces a groove at the central part of the lower surface of the nerve, 
so that its substance is displaced to both sides, assuming the shape of a 
kidney. If the process is very slow and gradual and the anatomical 
conditions are such that the nerve has sufficient space to expand on 
both sides, this transfiguration may reach a considerable degree without 
impairing the function of the nerve. The optic nerve behaves in this 
respect less like a nerve than — what it really is — a part of the brain. 
It is well known what great endurance the substance of the brain is 
apt to offer to a slowly growing tumor, that sometimes a growth may 
reach an enormous size without destroying the brain-substance, merely 
displacing and deforming it. 

Inasmuch, however, as there is a bony wall at the opposite side of 
the pressure, and as the space within the canal is extremely limited, 
the optic nerve has only a small chance of escaping injury, and, espe- 
cially if the changes in the artery occur a little more rapidly, a pro- 
gressive atrophy of the nerve is unavoidable. 

At first there may be only a slight atrophy in the centre, corre- 
sponding to the impression of the artery. In cases of this kind the 
nerve appears to be divided into two halves, connected by a white 
bridge, which is formed by compressed septa and atrophic fibres. But 
gradually the atrophy may progress until complete blindness occurs. 

According to the position of the ophthalmic artery, the compres- 
sion of the nerve, instead of taking place in the centre, may occur more 
laterally, so that only a small portion of the nerve is cut off, the larger 
part being displaced to the other side, filling the empty space between 
the arteries and the margin of the canal in a triangular shape. 

The ophthalmoscopic picture of the atrophic nerve may, therefore, 
vary greatly, according to the relation between the artery and the nerve 






468 THE EYE AND NERVOUS SYSTEM 

and the intensity and rapidity of the increasing pressure. The atrophy 
may be confined to one group of fibres, like the " crossed/' the " non- 
crossed/' or the " papillo-macular" fibres, and in some cases it might 
be possible to recognize approximately the seat of the pressure from the 
fibres involved in the atrophic process. 

The prolongation of the bony part of the optic canal within the 
cranial cavity is formed by a ring of thick, fibrous connective tissue. 
This ring is covered by the dura mater, which near the optic foramen 
forms a tensely stretched free margin. The base of the intracranial 
portion of the canal is formed by the internal carotid artery. If, 
therefore, this artery becomes hardened and considerably enlarged in 
diameter, it may push both optic nerves upward and forward against 
the hard margin of the dura mater, crushing and destroying both 
nerves. 

A sclerotic process in the carotid and in the vessels of the circle of 
Willis is, of course, apt to produce atrophy of one or both optic nerves 
by exerting pressure in the region of the chiasma. It is evident that 
eventually under suitable conditions the various forms of hemianopsia 
may be produced by a corresponding pressure. 

So, then, we see that sclerosis of the cerebral arteries may affect 
the optic nerves in numerous ways. We may have partial or com- 
plete blindness in one or both eyes ; we may have a narrowing of the 
visual field or central scotoma; we may have hemianopsia or ambly- 
opia. Any of these conditions may occur during the course of a general 
arteriosclerosis, and most cases of so-called " spontaneous optic atrophy 
of old age" are evidently due to a sclerotic process in the cerebral 
arteries. 

Arteriosclerosis has been regarded as a cause of quite a number of 
diseases of the eye. We will only mention these conditions here. 
Michel and his pupils have held that cataract can be produced by 
sclerosis of the carotid artery. According to some authors, glaucoma 
is produced by a general arteriosclerosis and the consequent disturbances 
in the circulation. Embolism of the central artery may be due to a 
sclerosis of vessels belonging to quite a different region of the body. 
A disease which only recently has been described more accurately, and 
which is supposed to stand in close relation to arteriosclerosis, is " reti- 
nitis circinata." 

There can be no doubt that arteriosclerosis forms one of the most 
important etiological factors in the production of diseases in general, 
and the more our knowledge of this subject advances the more we will 
appreciate this important fact. 



MULTIPLE SCLEKOSIS 469 

MULTIPLE SCLEROSIS; PSEUDOSCLEROSIS; 
DIFFUSE SCLEROSIS. 

The optic nerve is affected in nearly 50 per cent, of all cases of 
multiple sclerosis, and, if we consider that the changes in the optic 
disk may constitute one of the earliest manifestations of the disease, or 
may even precede all other symptoms by several years, we will readily 
appreciate the great diagnostic value of the ophthalmoscopic findings 
in this disorder. 

In order fully to understand the nature of the various affections 
of the optic nerve in multiple sclerosis, we must always bear in mind 
the fact that the optic nerve is not a nerve in the same sense as the 




Fig. 1.— Multiple sclerosis, showing a sclerotic area in the oculomotor nucleus. (Case of Dr. Spiller.) 

other peripheral nerves, but forms a part of the brain, so that it would 
be much more appropriate to speak of the optic tract rather than of the 
optic nerve, even in its most peripheral parts. 

The optic nerve represents a system of neurones, and corresponds 
in every respect to other systems, such as the posterior columns or the 
pyramidal tracts, and its affections in the various diseases of the cen- 
tral nervous system are entirely analogous to the affections of the other 
systems. In locomotor ataxia, for instance, the system becomes affected 
as such, as a unit. The posterior columns degenerate as a system, and, 
if the optic nerve is affected at all, it degenerates also as a system, under- 



470 THE EYE AND NERVOUS SYSTEM 

going complete atrophy. Multiple sclerosis, however, is not a systemic 
disease. No system is affected as such. The multiple plaques are dis- 
tributed indiscriminately through the entire brain and cord, regardless 
of any order or system. One plaque may reach from one system to the 
other, destroying a little here and a little there. The posterior col- 
umns may be affected at various levels, but they are not apt to be either 
more or less affected than any other system. According to this character 
of the disease, every system is more or less affected in multiple sclerosis, 
but no system is affected entirely throughout its course as is the case in 
the systemic diseases. 

The optic atrophy in multiple sclerosis is, therefore, very different 
from that in locomotor ataxia. In the latter disease the affection is 
symmetrical, it progresses slowly but surely, and ends invariably in 
complete atrophy of both nerves. The atrophy is from the start 
equally distributed over the entire disk. In multiple sclerosis, on the 
other hand, the atrophy is hardly ever complete. There is either a 
partial atrophy distributed equally over the entire disk or a partial 
atrophy confined to its temporal half. The affection in a large per- 
centage of cases remains one-sided. The morbid process may reach a 
certain stage and then remain stationary for a long time. The atrophic 
process never produces complete blindness in multiple sclerosis. On 
the contrary, an improvement has been reported in 50 per cent, of the 
cases. Occasionally even a restitutio ad integrum has been observed. 

In some cases there was an optic neuritis either one-sided or bilat- 
eral. Sometimes the optic atrophy shows traces of a previous neuritis, 
so that it can be assumed that in a certain percentage the atrophy is a 
secondary condition to a primary neuritis. 

The ophthalmoscopic finding often seems not to be in accordance 
with the real condition of the optic nerve. On the one hand there are 
cases in which vision seems to be considerably impaired in spite of 
an absolutely negative ophthalmoscopic picture, and on the other hand 
the disk may appear considerably atrophied and still the power of 
vision not be diminished in a degree which one would expect. 

This peculiarity, as well as the other irregularities described above, 
can easily be explained by the anatomical nature of the disease. A 
plaque may destroy considerable substance of the nerve without affect- 
ing the parts in the immediate neighborhood of the retina, thus offering 
the clinical picture of a retrobulbar neuritis. On the other hand, an 
affection of the retinal part of the optic nerve does not indicate its 
entire condition, as it does in other cases of atrophy. 

Of clinical significance in optic atrophy of multiple sclerosis is the 



MULTIPLE SCLEROSIS 471 

irregular, atypical behavior of the visual field. There is no homo- 
geneous narrowing, as in cases of tabes, but a most irregular constric- 
tion of the peripheral line. There may be a central scotoma or a com- 
bination of this with an irregular narrowing of the field. In a few 
cases an annular-shaped scotoma has been found, and in other rare 
instances small parts of the periphery were alone preserved while the 
function of all the rest of the nerve had been lost. Sometimes there is 
a central scotoma only for colors, or in some cases a general achroma- 
topsia. 




Fig. 2.— Multiple sclerosis of the optic nerve. (Case of Dr. Spiller.) 

Paralysis of the muscles of the eye is a comparatively frequent 
•symptom in multiple sclerosis, the various statistics showing an aver- 
age of about 20 per cent. The nature of the paralysis is always that of 
a central lesion, characterized by a conjugate paralysis, paralysis of 
convergence, and the impairment of isolated muscles. 

The paralysis itself is mostly an incomplete one, and its clinical 
course is characterized by manifold changes, such as improvements, 
exacerbations, intermissions, and relapses. The irregularity and the 
atypical course, therefore, correspond exactly to the pathological condi- 
tion of the optic nerve. The latter differs, however, from the nerves of 
the eye-muscles by its frequent peripheral lesions, the other nerve- 
trunks being affected only very rarely in multiple sclerosis. Here again 
the optic nerve behaves like a part of the brain, differing also in this 
respect from all the true peripheral nerves. 

Paralysis of the abducens seems to be the most frequent affection. 
Then follows the oculomotorius, which, however, is always affected onlv 



472 THE EYE AND NERVOUS SYSTEM 

partially and incompletely ; a complete paralysis of all branches of the 
oculomotorius, such as is seen in affections of the trunk of this nerve, 
hardly ever occurs in multiple sclerosis. Of the conjugate movements 
those to right and left are more frequently affected than the vertical 
movements of the eyeball. 

There may be disturbances in the movements of convergence. In 
cases of this kind the recti externi appear impaired in this function, 
while in other functions, such as the conjugate lateral movements, 
they seem to be entirely intact. This shows very plainly the central 
nature of the lesion. 

Ptosis has been observed in quite a number of cases, but, like the 
other affections, offers the character of incompleteness. It is more fre- 
quently unilateral than bilateral, and is often associated with double 
vision or other disturbances of sight. 

Partial paralysis and disturbances in the function of the eye mus- 
cles may be produced by cortical, subcortical, nuclear, and fascicular 
lesions, according to the localization of the sclerotic plaques, and we 
must admit that in a great many instances we are still unable to localize 
the lesion accurately from its clinical manifestations. 

A symptom to which a great diagnostic importance is generally 
attached in multiple sclerosis is nystagmus. Here we have to distin- 
guish between the nystagmus proper, a condition in which the eyeballs 
are in constant motion, and the so-called " intentional nystagmus," or 
"nystagmus of motion," in which the oscillatory movements do not 
take place when the eyeballs are at rest, but only when they are turned 
towards an object, especially at the end of the horizontal movements. 
It is the latter form which we find most frequently in multiple 
sclerosis. Inasmuch, however, as slight oscillatory horizontal motions 
of the eyeballs after a sudden extreme lateral movement may occur 
also in hysterical or even normal individuals, we have to be very careful 
not to attach too much importance to this symptom, or at least confine 
its diagnostic value to the more marked cases. Sometimes the twitch- 
ing of the eyeballs takes place only with the horizontal motions, while 
in other cases every motion, even vertical ones, may be accompanied by 
this phenomenon. 

Quite a number of theories have been offered to explain this peculiar 
symptom of the disease, of which we will mention only the most 
important. 

Some authors regard the nystagmus of multiple sclerosis analogous 
to the intentional tremor of the extremities, due to a paralysis of the 
centres of association or to an affection of the peripheral nerves. Others 



MULTIPLE SCLEROSIS 



473 



explain the nystagmus by a disturbance in the equilibrium, due to the 
insufficiency of certain groups of muscles. According to an other theory 
the nystagmus is produced by a diminution of the tonus of the muscles, 
the innervations from the centres being interrupted and impaired by 
the various sclerotic lesions. 

Whatever the true cause of the nystagmus may be, so much is cer- 
tain, that it is located in the central nervous system, in the brain or in 
the medulla oblongata. The various territories, which have been 
claimed to be responsible for this phenomenon, are still very numerous, 
and in all probability we have to deal here with a condition which can 
be brought about by manifold lesions in the brain. 





Fig. 3.— Multiple sclerosis of the optic chiasm and one optic nerve. (Case of Dr. Spiller.) 



The function of the pupils remains normal, as a rule, throughout 
the entire course of the disease. If, however, we bear in mind the ana- 
tomical picture of multiple sclerosis, if we consider that sclerotic 
plaques are apt to occur in any part of the central nervous system, we 
will readily understand that there is really no symptom in neuropath- 
ology which may not occasionally occur in this disease. Abnormal con- 
ditions of pupils, therefore, have been reported, but they really do not 
belong to the characteristics of the disease, and if they do occur, they 
mislead rather than assist in diagnosis. The pupillary symptoms, 



474 THE EYE AND KEKVOUS SYSTEM 

which were observed in very rare cases, were: myosis, difference of 
pupils, diminution and exaggeration of light-reflex, loss of light-reflex, 
loss of reflex on accommodation and paradoxic reaction. 

There is hardly any other disease of the central nervous system 
in which the clinical phenomena of the eye are so characteristic of the 
pathological process, and in which, therefore, the symptoms are of the 
same significance as in multiple sclerosis. 

There is a disease, the clinical manifestations of which resemble 
multiple sclerosis, but wherein the anatomical changes do not corre- 
spond to the clinical symptoms. This affection was first described by 
Westphal under the name of pseudo-sclerosis, and recently Struempell 
has thrown more light on this peculiar subject. It is of special im- 
portance to us, inasmuch as the condition of the eye forms one of the 
main factors in the differential diagnosis between this affection and 
genuine multiple sclerosis. While the other symptoms offer a great 
resemblance to the clinical features of multiple sclerosis, the eye symp- 
toms always remain absent in pseudo-sclerosis. There is no optic 
atrophy, no muscular palsy, no nystagmus. 

Another affection, which offers many clinical resemblances to mul- 
tiple sclerosis, pseudosclerosis and general paresis, is called diffuse 
cerebral sclerosis. This is a disease which may occur at all ages, in 
children as well as in adults. The main symptoms consist of spasticity 
of lower and upper extremities, increased reflexes, mental apathy and 
dementia and various palsies which may increase to complete paralysis. 
This disease also is characterized by the absence of any ocular symp- 
toms, optic neuritis having been observed only in a single case 
(Heubner). 

FRIEDREICH'S DISEASE. 

In spite of the general interest which was created by Friedreich's 
first publications and which has prevailed ever since, in spite of the 
numerous excellent clinical observations and anatomical investigations 
of this disease, we are still far from having reached a generally accepted 
view regarding the nature and pathology of this affection. Even with 
reference to the clinical features of the disease, there still exist great 
differences of opinion, especially as to the grouping into various types. 

As far as ocular symptoms were concerned, it was emphasized by 
Friedreich himself, that the disease is characterized by a complete 
absence of any abnormalities or disturbances in the eyes, with the 
exception of one symptom, namely, a certain form of nystagmus. 

This nystagmus, which is sometimes called pseudo-nystagmus, or 



FKIEDKEICH'S DISEASE 475 

atactic nystagmus, does not consist of the rhythmical oscillations of 
the eyeball like the ordinary nystagmus, regardless whether the eyes are 
at rest or whether movements of some kind are performed; it consists 
of irregular twitchings, which occur only when the eyes are fixed on a 
moving object, especially in the horizontal direction. When the eyes 
are at rest, or when they are fixed at a point in the sagittal plane, they 
are as a rule perfectly quiet. This form of nystagmus, which resem- 
bles that of multiple sclerosis, only being more intense than the latter, 
forms one of the most constant phenomena of Friedreich's disease. 
Only very rarely is this symptom absent ; but then it can sometimes 
be produced artificially by turning the patient several times around 
his axis. In all probability this j)henomenon has to be considered as a 
disturbance in co-ordination, as an atactic condition of the muscles of 
the eye. 

As said before, this was claimed by Friedreich to be the only ocular 
symptom of the disease, and up to the present day a great many authors 
regard all other abnormalities of the eyes as not belonging to this 
affection, and therefore as a contraindication to the diagnosis. Inas- 
much, however, as soon afterwards a considerable number of cases were 
reported, which did offer ocular symptoms and differed also in other 
respects from the cases described by Friedreich, Pierre Marie described 
another clinical picture under the name of heredoataxia cerebelleuse 
(hereditary cerebellar ataxia). This disease, which had a great many 
traits in common with Friedreich's disease, such as the familiar char- 
acter, the ataxia and progressive nature, differed from it by the condi- 
tion of the patellar reflexes, which were increased here and absent in 
Friedreich's disease, and also in the symptoms of the eye, muscular 
palsies, loss of pupillar reflex, disturbances of vision, various affections 
of the optic nerve being comparatively frequent in this disease. 

A theory had been offered according to which Friedreich's disease 
consisted of a maldevelopment of certain parts of the spinal cord, while 
the affection described by Marie was due to an abnormal cerebellum. 

The attempt to keep these two types of the disease apart has not 
been successful. The more observations were put on record, and the 
more attention was given to this interesting disease, the more it was 
realized that such a division is more or less arbitrary. It soon became 
necessary to regard some cases as a " mixed type," as they offered 
features of each group in equal proportion. Some cases were diagnosed 
as Friedreich's disease by one observer and as cerebellar ataxia by 
another. In a few instances both diagnoses had to be made among 
brothers, one resembling the one and the other the other type. 



476 THE EYE AND NERVOUS SYSTEM 

Even anatomically it was not possible to keep the two types apart. 
In some cases both the cerebellum and the spinal cord were affected. 
In others the anatomical findings seemed not to correspond to the 
clinical features. According to one theory the disease always consists 
of a spinocerebellar affection, the tracts communicating between the 
cord and the cerebellum in both directions being affected. 

The division of the disease into these various types will in all prob- 
ability not withstand future investigations. All that we can say to-day 
is, that clinically as well as anatomically the features of the disease may 
vary, and that we must not draw the line too closely in making the diag- 
nosis, but rather allow a certain latitude, even if the clinical picture 
differs from the original description of Friedreich. 

As far as the eye-symptoms are concerned we may say that the 
special form of nystagmus, which was described above, is by far the 
most frequent and constant symptom of the disease, but that, in some 
cases it may be absent. Ocular palsies, optic neuritis, and even the 
Argyll-Robertson pupil may occur in the disease, although they are 
comparatively rare. 

PARALYSIS AGITANS. 

Ocular symptoms do not form an essential feature of paralysis agi- 
tans. There are a few cases on record in which paralysis agitans was 
combined with other diseases of the central nervous system, as, for 
instance, locomotor ataxia. The loss of pupillar reflexes or affections 
of the optic nerve in cases of this kind would, of course, not form a 
feature of paralysis agitans, but of the complicating disease. 

Inasmuch, however, as in paralysis agitans the muscular apparatus 
of the entire body is apt to be affected in the characteristic manner, it 
is evident that the muscles of the eye may once in a while show the 
same morbid condition, although as a rule they escape even this affection. 

The characteristic condition of the muscles consists of the typical 
rhythmical tremor, and of a very marked tension, which interferes with 
normal motion, so that in the later stages the individual may become 
nearly unable to perform any movements at all, although there is no 
true paralysis in the ordinary sense. The main difficulty in performing 
motions adequate to their purpose seems to arise from the inability of 
the patient to contract a group of muscles suddenly after .they are at 
rest, or to relax them quickly when they are contracted. Both these 
functions are indispensable to normal motion. This condition would 
also explain the inability of the patient to arrest the body suddenly 
when in locomotion, a symptom known by the names of propulsion, 



PAKALYSIS AGITANS 477 

retropulsion and lateropulsion. If a person, who is walking, wants 
to arrest this locomotion suddenly, he must quickly contract the antag- 
onistic muscles, must throw the trunk in the opposite direction, a func- 
tion which an individual afflicted with paralysis agitans is unable to 
perform. 

If now we apply this condition of the muscular apparatus to the 
muscles of the eye, we would expect quite a number of interesting phe- 
nomena. To the tremor would apparently correspond a nystagmus. 
But this symptom has never been observed in paralysis agitans. This 
fact furnishes another proof for the assumption that nystagmus is pro- 
duced by some central lesion and not by a disturbance in the general 
muscular tonus, because if the latter view were correct we would be 
bound to observe this symptom at least occasionally in paralysis agitans. 

The tension and rigidity do occur in the ocular muscles, although 
far less frequently than one would expect. The sphincter is never 
affected; the pupils have always been found normal in this disease. 
Likewise the optic nerve always remains unimpaired. The rigidity is 
very apt to show itself in the orbicularis, thus interfering with the 
mimical motions, the expression of the features, and therefore contribu- 
ting to the mask-like appearance of the face. The closing and opening of 
the eye is sometimes very slow and sluggish, and in a few cases there 
was a partial ptosis. In order to assist in the opening of the eyes, the 
musculi frontales are contracted, and on account of the difficulty in 
relaxing the muscles, patients often have marked wrinkles in the fore- 
head. 

The movements of the eyeballs are sometimes somewhat retarded. 
A few cases have been observed where there was great difficulty in con- 
vergence ; also a spasm of accommodation has been described. In one 
case Graefe's symptom occurred on one side in the hemilateral form 
of the disease. 

There is only a single case (Minkowski) on record in which there 
was a complete ophthalmoplegia externa. Both eyes were entirely 
closed and could not be opened by the patient. When one raised the 
lids with the finger, the eyeballs stood in a parallel axis, directed straight 
forward, without ability on the part of the patient to move them in 
any direction. 



CHAPTER XII. 

PARASYPHILITIC AFFECTIONS, INSANITIES, AND TOXIC 
ENCEPHALOPATHIES. 

By F. X. DERCUM, M.D. 

PARESIS. 

The ocular phenomena of paresis consist in disturbances which may 
involve all parts of the visual apparatus, — namely, the muscles, internal 
and external, the optic nerve, and the fundus. These disturbances must 
be studied in detail, but, in order that we should do so intelligently, it 
will be necessary, first, to enter upon certain general considerations. 
Paresis is an affection which begins, as a rule, insidiously and extremely 
gradually. Its earliest symptoms are usually those of a purely func- 
tional disturbance, and occasionally this disturbance not only precedes 
the appearance of physical signs, but antedates the latter by a consider- 
able period of time. During this period the symptoms may, roughly 
speaking, resemble those of neurasthenia, and the differential diagnosis 
between the two affections is attended with considerable difficulty and 
may indeed be open to serious doubt. Paresis, we may here remark, 
is readily divided, as regards its course, into three stages, — first, the 
initial or prodromal period ; second, the period of the fully developed 
disease, and, third, the period of profound dementia — the terminal 
period. During the second or third stages no difficulty with regard to 
diagnosis can possibly arise as each of these stages is attended by physical 
signs, both positive and numerous. It is in the first stage only, as just 
stated, that difficulty may arise. If, therefore, the existence of an early 
physical sign can be determined, a vast assistance is given to the prob- 
lem. Indeed, the appearance of such a symptom assumes a decisive 
importance. 

Paresis, we should remember, is a degenerative disease attended by 
a constantly increasing loss of function. One of the cardinal points 
to be borne in mind in its early diagnosis is that the changes in func- 
tion are quantitative and not merely qualitative. In its very begin- 
nings are these fundamental features to be noted. There is a general 
though slight mental enfeeblement, an inability to apprehend readily,. 
a faint but unmistakable loss of memory, slight lapses in conduct, and 
a neglect of the proprieties. There is especially an evident inability 
478 



PAEESIS 479 

of the patient to properly appreciate his surroundings ; his close con- 
tact with his environment is lost, and this is true not only as regards 
his relation to matters of business and to his family, hut also as regards 
his own illness. This blunting or obtusion of the mental faculties is 
the early and often faint expression of the more profound dementia 
to supervene in the future. In neurasthenia, on the other hand, the 
character of the mental operations never changes; but one cardinal 
symptom is noted, — namely, the diminution in the capacity for sustained 
mental effort. There are never, however, any losses of function. There 
is no loss of memory, no blunting of judgment, no obtusion of the moral 
and aesthetic faculties — none of those countless changes in conduct and 
habits to indicate a progressive psychic disintegration. 

Notwithstanding the fact that such fundamental differences obtain 
in the symptomatology of neurasthenia and of early paresis, it is at 
times a matter of extreme difficulty in practice to arrive at a decision, 
so faint and shadowy may the beginning symptoms be. It is obviously 
of vast importance to discover actual physical signs at the earliest 
moment. Unfortunately, it happens that at this stage the pupils and 
eye-grounds often fail to yield us any information. One sign, however, 
that may be present and one which I personally regard as of great 
significance, is the existence of a slight degree of amblyopia. With or 
without a diminution of the color sense, it is an invaluable symptom. 
It may antedate distinct and demonstrable anomalies of the pupils or 
changes in the eye-grounds. Visual hypsesthesia is apparently in 
keeping with the general psychic obtusion that is setting in, as well as 
with the lessening of general cutaneous sensibility noted sooner or later. 
It is a sign whose importance is not sufficiently recognized, nor is it sought 
for in individual cases as it should be. The difficulty with regard to 
light perception is not always slight in the beginning, but may amount 
to an actual loss of vision, though this early loss is frequently transient 
in duration. Further, it is admitted by numerous writers — Mendel, 
Mickle, Kraepelin, Ziehen, Ballet, and Blocq — that optic atrophy may 
be the first noticeable symptom of paresis. 

The most common of the ocular phenomena of paresis are those 
which relate to the pupil. One of the earliest phenomena usually ob- 
served is that of inequality of pupils. Difference in the size of the 
pupils, we should remember, may occur physiologically, but in such 
case this difference is usually slight. If, however, a difference in the 
size of the pupils be noted, and this difference be associated with a slug- 
gishness to light reaction or if the pupils, being equal, react slightly 
differently to light, the finding is suggestive with regard to paresis in 



480 THE EYE AND NERVOUS SYSTEM 

its incipiency. In the early stages of paresis, indeed, a difference in 
the promptness of the response to light reaction between the right and 
left pupils is almost the rnle. Likewise an impairment of the consen- 
sual light reaction may be the first symptom of a pupillary disorder 
in paresis, and it is of the same value as a difference of reaction of the 
two pnpils. 

The above facts are established by numerous observations and admit 
of no question. How valuable slight changes of the pupils are in enabling 
us to make a diagnosis is revealed by a case reported by Lucas in which 
a diagnosis of melancholia had been made, but in which JofTroy after- 
wards noted inequality of the pupils and suspected a paresis, which later 
matured. 

Narrowing or small size of the pupils, even if slight, taken together 
with other symptoms, is very significant. Distinct myosis is, of course, 
a symptom of unquestioned value. Exceptionally, it occurs in the 
very early stages of paresis. Changes in the fundus of the eye may 
also be noted early, though such changes are exceptional. However, 
they have been noted early by Walker and more especially by Knapp. 
Knapp, indeed, has reported three cases of paresis in which optic 
atrophy preceded the mental symptoms. Abolition of the light reflex 
with preservation of accommodation — the Argyll-Robertson pupil^ 
may also infrequently be observed in the early stage of paresis, though 
this is quite unusual. Commonly, sluggishness of the light reaction 
alone is noted in the early stage. 

The mental changes of paresis may long antedate the appearance 
of physical signs. However, this is not by any means the rule. Physi- 
cal signs may indeed make their appearance either simultaneously with 
the mental changes or may sometimes even precede the latter. Notably 
is this the case w T ith the visual phenomena. In other words, it is un- 
usual, in practice, to meet with early cases of paresis in which some 
ocular changes, even though slight, cannot be found. 

The relative frequency of ocular phenomena in the early part of 
the disease may be gathered from the statistics of JofTroy. JofTroy 
examined two hundred and twenty-seven cases of paresis, of which the 
majority were in the first period of the disease. Two hundred and 
twelve of these cases presented ocular symptoms. JofTroy believes that 
among the fifteen who did not have ocular symptoms a later examina- 
tion, at a more advanced stage, would certainly have revealed them. 

If a systematic study be made of the eyes in paresis in the stage 
of the well established disease, the following phenomena are noted. 
Eirst, there is very frequently observed an inequality of the pupils. 






PAKESIS 481 

Some idea of the relative frequency of this symptom can be gained 
by the following statistics which have been taken in part from the 
groupings of Mignot and of Bumke. Siemerling noted inequality in 
26 per cent, of the cases examined by him, Lasegue 33 per cent, Fran- 
cotte 51 per cent., Moreau 58 per cent., Mendel 60 per cent, Mobeche 
61 per cent., Kaes 62.7 per cent, Marie 63 per cent., Oebecke 64 per 
cent., Benaud 65 per cent., Seifert 66 per cent, Boy 66 per cent, 
Begis 66 per cent., Doutrebente 77 per cent., and Vincent 80 per cent. 
Mignot found 64 per cent. ; Cololian, who studied fifty-seven cases, 
found unequal pupils in almost all of them. Dawson found 92.3 per 
cent, in the forty cases studied by him. It was the most frequent of 
all the ocular phenomena in his cases. The right pupil was the larger 
in twenty-five, the left in eleven. Kornf eld and Bikeles found unequal 
pupils in 70 per cent, Baehlmann in 60 per cent., Gudden in 57.5 per 
cent, Wallenberg in 53 per cent., A. Westphal in 45 per cent., Hillenberg 
in 45 per cent, and Siemerling in 27.5 per cent JofTroy found inequal- 
ity of the pupils in one hundred and forty-four of his two hundred and 
twenty-seven cases — that is, 63.4 per cent. If to these there are added 
lis cases of extreme double mydriasis and extreme double myosis, he ob- 
tains 87 per cent, of cases of modifications of the dimensions of the iris. 

Variations in the frequency with which unequal pupils are observed 
is, in part, to be attributed to the stages at which the cases have been 
examined and to a less degree to the element of personal equation : some 
observers noting minute differences and others only inequalities that 
-are striking. Notwithstanding the divergence in the .percentage ob- 
tained by different observers, it cannot but be admitted that inequality 
of pupils is an exceedingly common symptom of paresis. However, of 
itself inequality of the pupils has no more than a confirmatory value 
in the diagnosis. As is well known, it may occur in gross organic dis- 
ease, brain syphilis, and the insanities; indeed, it may in moderate 
degree be occasionally noted in perfectly normal individuals. It is 
important to add that the degree of inequality in paresis may vary 
decidedly in a given case, and sometimes during a comparatively short 
period of observation. Bapid fluctuations are by no means infrequent. 

The pupil in paresis is not infrequently irregular in shape, — that 
is, it may be slightly oval or ovoid or its circumference may be made 
irregular by various modifications of shape. These modifications do 
not, however, lead to gross deformities; notwithstanding, they can be 
very readily observed. They are undoubtedly present in a percentage 
of cases. Kaes found them in 3.1 per cent., Mobeche in 61 per cent., 
and Mignot in 18 per cent. Dawson noted irregularity in outline in 
31 



482 THE EYE AND NERVOUS SYSTEM 

one or both pupils in thirty-seven out of thirty-nine cases (94.8 per 
cent.). JofTroy noted in one hundred and twenty-five cases, in which 
the symptom was studied, deformity of both pupils in ninety-three, — 
that is, 75.2 per cent. In eight cases one pupil only was deformed. 
Mignot observed deformities in fifteen cases out of twenty-two. In 
seven of his cases Mignot observed the gradual diminution and final 
loss of the pupillary reflexes. JofTroy observed irregular pupils in two 
cases in which the symptom was not accompanied by impairment of 
the light reflex. However, in these two cases he later observed, first, 
paresis and then paralysis of the light reflex. Beginning deformity 
or irregularity of the pupil is to be regarded as a precursor of the 
Argyll-Robertson sign. In one case inequality made its appearance 
while the patient was under observation at the hospital. In another, 
pupillary disturbances made their appearance fifteen days after 
admission. 

Myosis is also a symptom very frequently observed in the well- 
established period of the disease. The opposite condition, mydriasis, 
likewise occurs, and would appear from the following figures to be 
decidedly less frequent than myosis. 

Kaes found myosis in 7.2 per cent., Mobeche in 12 per cent., Doutre- 
bente in 12 per cent., Moreau in 18 per cent., Vincent in 38 per cent., 
and Marie in 39 per cent. Mignot found, out of twenty -two cases, 
myosis in six. Dawson found myosis on both sides in six cases out 
of twenty-three ; in three cases he noted myosis in only one eye. 

As regards mydriasis, Kaes found it in 3.6 per cent., Doutrebente 
in 4 per cent, Vincent in 14 per cent, Mobeche in 15 per cent., Moreau 
in 26 per cent., and Marie in 44 per cent. Out of twenty-two cases 
Mignot found mydriasis in only one. Out of twenty-three cases Daw- 
son found mydriasis in eight In one case he observed mydriasis of 
the right eye and myosis of the left Mydriasis is a paralytic phe- 
nomenon which Fournier regards rather as a symptom of syphilis than 
of paresis. That it should, at times at least, be regarded as an expres- 
sion of ophthalmoplegia interna and indicative of nervous syphilis 
rather than of paresis seems plausible, but involvement of the intrinsic 
muscles of the eyes is so frequent in cases of undoubted paresis that 
this explanation can apply only exceptionally — perhaps in cases in 
which true syphilitic processes are going on at the same time as the 
degenerative changes of paresis. 

Next most important are changes in the light reflex. This is im- 
paired or lost upon one side or both in a very large number of cases. 
In statistical studies of the loss of the reflex, different observers have 



PARESIS 483 

likewise arrived at different percentages of results, as the following 
figures (grouped by Alignot) will show: Oebecke 26 per cent., Thom- 
sen 47 per cent., Mendel 47 per cent,, Siemerling 54 per cent, ALagnan 
and Serieux 60 per cent., Moeli 61 per cent., Siemerling (second sta- 
tistic study) 64 per cent., Erancotte 88 per cent., Siemerling (third 
statistic study) 68 per cent., and Vincent 90 per cent. To these should 
he added Dillenberg 47.5 per cent, Wollenberg 50 per cent., A. West- 
phal 50 per cent., Gudden 41.2 per cent., Kornfeld and Bikeles 62 
per cent., and Hirschl 50 per cent. JofTroy noted loss or impairment 
of the light reflex in one hundred and seventy-one cases out of the two 
hundred and twenty-seven cases studied by him, — that is, in 75.3 per 
cent. It was abolished on both sides in one hundred and three cases, 
abolished upon one side and diminished upon the other in fourteen 
cases, diminished upon both sides in thirty-five cases, abolished upon 
one side and normal upon the other in nine cases, and diminished upon 
one side and normal upon the other in ten cases. 

M. de Montyel has made an exhaustive analysis of the light reflex. 
He found it normal in 30.8 per cent., abnormal in 69.2 per cent. Exag- 
geration was present in 5.3 per cent., diminution in 43.1 per cent, 
loss in 47.6 per cent. Analyzing his findings with regard to the period 
of the disease, he found the reflex exaggerated in 17.4 per cent during 
the first period, and 2.3 per cent, in the second period, and never 
during the third period. He found it diminished in 44.6 per cent, in 
the first period, 47.2 per cent, in the second period, and 34 per cent, 
in the third period. He found it lost in 32.5 per cent in the first 
period, 45 per cent, in the second period, and 66 per cent, in the third 
period. He found it normal upon one side and lost on the other in 
5.5 per cent, in the first period, 5.5 per cent in the second, and never 
in the third period. Analyzing his results with regard to the form of 
paresis, he found it abnormal in 66.1 per cent, in the expansive form, 
in 100 per cent in the depressive form, in 45.4 per cent, in the mixed 
form, in 80.6 per cent, in the simple demented forms, and in 25 per 
cent, of cases with remission. 

Dividing his cases into those which were agitated and those which 
were quiet, he found it abnormal in 69.2 per cent, in the agitated forms 
and 75.8 per cent, in the quiet forms. Analyzing his results again as 
to ages, he found the light reflex abnormal in 93.8 per cent, in the 
ages from twenty to thirty, in 63.9 per cent, in the ages from thirty 
to forty, in 67.2 per cent, in the ages from forty to fifty, and 70 per 
cent, in the ages of fifty to sixty. De MontyeVs conclusions from these 
studies were as follows : First, the light reflex was move frequently 



484 THE EYE AND NERVOUS SYSTEM 

abnormal than normal. Second, exaggeration of the reflex was excep- 
tional. Third, there was only a slight difference in the frequency of 
the occurrence of the diminished and the lost reflex. Fourth, the 
changes in the reflex were identical on the two sides ; only exceptionally 
was the reflex lost upon one side and normal upon the other. Eifth, 
in the first period the normal reflex was more frequent than the abnor- 
mal, while the reflex is more frequently abnormal in the second and 
third periods. Sixth, exaggeration and unequal changes on the two 
sides were always present in the initial stages of the disease; the 
frequency of loss was proportionate to the progress of the disease. 
Diminution was more frequent than loss during the first period; the 
proportion was about equal in the second and reversed in the third 
period. Seventh, the reflex was abnormal in one-fourth of the remis- 
sions. Eighth, exaggeration was seen only in the expansive and mixed 
forms. Loss of the reflex prevailed over diminution in the demented 
form; only in the demented form were both eyes unequally affected. 
Ninth, an abnormal reflex was always noted in the traumatic cases. 
Tenth, at all ages, the abnormal reflex was more frequent than the 
normal; however, abnormality of the light reflex was especially noted 
in the extreme ages, especially in the younger cases. Eleventh, in the 
first and second periods the reflex was more affected when the motor 
symptoms were more pronounced. (See Chapter VI, p. 308). 

The consensual light reflex should also be studied. Its importance 
is not fully appreciated. Dawson found absence of the consensual light 
reflex in one or both eyes in twenty-seven out of forty cases (67.5 per 
cent.). Further, it was involved without impairment of the direct 
light reflex in eleven cases, though in only two of these cases was it 
actually absent. Impairment or loss of the consensual light reflex must 
be regarded as of great significance whenever met with. Dawson con- 
cludes that it is more often affected than the direct light reflex. 

Next, it is of importance to study the reaction to accommodation. 
This is preserved, as in tabes, long after the light reflex is lost. How- 
ever, it is sooner or later involved. The more advanced the case, other 
things equal, the more likely is loss or impairment of accommodation 
to be present. Mignot, in twenty-two cases, found accommodation to 
be normal in eleven, lost in five, and diminished in six. De Montyel 
made an elaborate study of the reaction in thirty cases. These cases were 
followed from the outset to the termination of the disease, the reflexes 
being examined, in all, some six hundred and eighty times. It was 
found normal in two hundred and eighty-one examinations, abnormal 
in three hundred and ninety-nine. In fifteen examinations, diminution 



PAEESIS 485 

was present in one hundred and sixty-eight and loss in two hundred 
and ten. It was normal on one side and abolished on the other in six 
examinations. 

In almost all the cases changes were present in both eyes simul- 
taneously. Only in 1.5 per cent, was the reflex normal upon one side 
and lost upon the other. The majority of cases presented a diminution 
of the reflex. When exaggeration was present it was always moderate. 
Only six times did the reflex show changes in one eye. Six times 
the loss of of the reflex of one eye coincided with abnormality of the 
other. Analyzing his results as to the various periods of the disease, 
de Montyel found that in the first period the accommodation reflex was 
normal in 63.2 per cent, abnormal in 36.7 per cent. During the second 
period these figures were reversed, for now he found them normal in 
only 33.7 per cent, and abnormal in 66.3 per cent. In the third period 
a still greater reversal of percentage took place. It was normal in 16.7 
per cent, and abnormal in 83.2 per cent. He found it exaggerated in 
the first period 15.3 per cent. He at no time found it exaggerated in 
the second or third periods. He found it diminished in 64.2 per cent, 
in the first period, in 41.9 per cent, in the second period, and 26.1 per 
cent, in the third period. He found it lost in 20.4 per cent, in the first 
period, 54.4 per cent, in the second period, 73.9 per cent, in the third 
period. In 3.5 per cent, he found it normal upon one side and abolished 
on the other in the second period of the disease. Analyzing those cases 
in which the reflex was found to be merely diminished and not lost, he 
found that it was somewhat diminished in 76.2 per cent, and very 
much diminished in 23.8 per cent, in the first period. During the 
second period he found that it was diminished in 88.6 per cent, and 
much diminished in 11.4 per cent. In the third period he found it 
diminished in 82.9 per cent, and much diminished in 17.1 per cent. 
Analyzing his results according to the depressive, the expansive, or the 
mixed forms, or forms with the remissions, he found the reflex normal 
in 53 per cent, and abnormal in 46.9 per cent, in the expansive form, 
normal in 51.7 per cent, and abnormal in 48.3 per cent, in the de- 
pressive form, normal in 53 per cent, and abnormal in 46 per cent, in 
the mixed form, and in the demented form normal in 25.3 per cent, 
and abnormal in 74.7 per cent. While in the cases with remissions 
he found this reflex normal in 62.5 per cent and abnormal in 37.5 
per cent. He also found that it was exaggerated in 15.2 per cent, in 
the expansive form and never exaggerated in the depressive, mixed, 
simple demented forms, and forms with remissions. It Avas diminished 
in 42.4 per cent, in the expansive form, in 100 per cent, in the depres- 



486 THE EYE AND NEKVOUS SYSTEM 

sive form, in 41 per cent, in the mixed form, and 33.3 per cent, in 
the simple demented form, and in 100 per cent, in the forms with 
remissions. It was lost in 42.4 per cent, in the expansive form, never 
entirely lost in the depressive form, lost in 58.8 per cent, in the mixed 
form, in 64 per cent, in the demented form, and never entirely lost 
in the form with remissions. It was normal upon one side and abolished 
on the other in no case of the expansive, the depressive, or the mixed 
form. This, however, occurred in 2.5 per cent, in the simple demented 
form, and in none of the cases with remissions. 

Analyzing his findings as to age, de Montyel found that the reflex 
was abnormal in 88.9 per cent, in the ages from twenty to thirty, in 
57 per cent, in the ages from thirty to forty, in 21 per cent, in the 
ages from forty to fifty, in 82.9 per cent, in the ages from fifty to 
sixty. His conclusions are as follows : First, that the accommodation 
reflex is more frequently normal than abnormal; second, that the 
exaggeration of the reflex is exceptional; third, that loss is more fre- 
quent than diminution ; fourth, in one-half per cent, it is abnormal upon 
one side and normal on the other; fifth, exaggeration or diminution 
are usually moderate ; sixth, only during the first period of the disease 
is the reflex more frequently normal than abnormal, and especially 
is this the case in the third stage ; seventh, exaggeration and unequal 
changes on both sides were noticed exclusively in the initial stages, 
while the loss of the reflex was in direct proportion and diminution 
of the reflex in indirect proportion to the progress of the disease ; eighth, 
an abnormal reflex is noticed in more than one-third of the cases in 
the period during the remission, and it was only in the demented form 
that the abnormality of the reflex prevailed over the normal reflex. In 
all of the other varieties — expansive, depressive, and mixed — the nor- 
mal was more frequent than the abnormal ; ninth, the reflex was more 
frequently and more profoundly changed in the period of excitement; 
tenth, changes in the reflex were most frequently noted in the extreme 
ages — before thirty and after fifty ; eleven, exaggeration of the reflex 
was present, while the motor disturbances were slight. During the 
first and second periods of the disease the reflex was the more pro- 
foundly changed the more the motor disturbances were marked. 

Changes in the eye-ground, with progressive amblyopia and amau- 
rosis, may be noted in paresis. However, as compared with tabes, 
such changes are relatively infrequent. Perhaps, as Klippel points 
out, this difference is due to the fact that in tabes the peripheral sen- 
sory neurone is primarily attacked, while in paresis the peripheral 
sensory neurone is but rarely and only indirectly attacked, — that is, by 



PAEESIS 487 

the propagation of degeneration from neurone to neurone. The change 
in the optic nerve, according to Keravel and Raviart, is more insular 
in tabes and more annular in paresis. However, lesions of the fundus 
occur in paresis earlier and more frequently than the older observers 
thought. That they sometimes occur at a very early period is proven 
by the three cases placed on record by Knapp. Thus, a man of thirty- 
seven presented a history of sudden loss of vision in 1889. This loss 
proved temporary, and vision soon returned. Five months later, how- 
ever, failure of vision set in, was progressive, and finally became com- 
plete. Optic atrophy was present in both eyes. In another case, a man 
of thirty-six, there was a gradual loss of vision with gray atrophy of 
both nerves. In a third case, a man of forty-five presented marked 
impairment of vision of both eyes, while both discs were bluish-gray. 
In all of these cases the eye-ground changes, according to the author, 
preceded the paresis. 

That fundus lesions occur more frequently than was formerly be- 
lieved is shown by the studies of Keraval. The latter has made a study 
of the fundus in paresis and in its initial lesions in fifty-one cases. He 
found that forty-two cases presented lesions of the fundi. In seven 
patients in an advanced stage of the disease there were five with white 
atrophy, one with gray atrophy, and one with sclerochoroiditis. In 
thirteen cases the papilla was pale. In twenty-two there was involve- 
ment only of segments of the papillae (preliminary stage of optic 
atrophy). In nine cases in the period of remission there were no fun- 
dus lesions. Raviart made a study of the fundus in forty-four cases 
of paresis. In one there was bilateral white optic atrophy. In ten 
both papillae were pale ; in two a wmite papilla on the right side ; in 
four there were gray papillae; in five the papillae were pale (or washed 
out) ; in six only were the fundi normal. Changes occurred therefore 
in 73.33 per cent. The majority of the cases in which these changes 
were observed were in the second stage of the disease. Visual acuity 
was slightly diminished in the majority of cases. 

Individual statistics differ considerably regarding the frequency of 
optic atrophy. Thus, in contrast to the above results are the findings of 
Hans Gudden. The latter found primary optic atrophy only sixty-five 
times in one thousand three hundred and twenty-six cases of paresis. 
Morselli again, on the other hand, thinks that optic atrophy is very 
common in paresis. Clifford Allbutt also found forty-one cases of 
atrophy in fifty-three of paresis, while Tebaldi found the fundus healthy 
in only one case out of twenty. Boy found optic atrophy in ten per 
cent., Mendel in five per cent., Joffroy in twelve per cent. Optic atrophy 



488 THE EYE AND NERVOUS SYSTEM 

sufficiently marked to cause blindness is relatively rare in paresis. 
Varying degrees of amblyopia are more common. Finally, optic 
atrophy, as we have already seen (page 480), may be the first physical 
sign of paresis. 

Disturbances of the visual fields may occur in paresis, but they are 
not common. This is especially true of marked disturbances. Con- 
tracture, however, may be noted, as, for instance, in a case reported by 
Deutsch of concentric narrowing of the fields. More rarely gross de- 
formities are observed, as in a case of Storch in which, after an epilepti- 
form seizure, a left hemianopsia was noted which persisted for a year. 

Among the physical signs of paresis are various palsies of the 
cranial nerves, more especially of the third, fourth, and the sixth pair. 
These may give rise variously to strabismus, diplopia and ptosis. They 
may make their appearance at the very beginning of paresis, or may 
occur during the period of the fully developed disease. Usually these 
palsies are not very pronounced or accentuated. Slight degrees of 
ptosis, weakness of adduction or abduction may be noted, though as a 
rule careful study is required to detect these anomalies. In this respect 
they differ markedly from those observed in tabes and syphilis. Quite 
commonly also they are transitory and fugacious. Dawson, in forty 
cases, noted palsies of external ocular muscles in only five; Raekes in 
18.2 per cent, ; Joffroy in 16.7 per cent. Out of his two hundred and 
twenty-seven cases, Joffroy noted ocular palsies thirty-eight times. In 
twelve cases a simple ptosis was noted ; fourteen times paralysis or pare- 
sis of muscles supplied by the third nerve and five times of the muscle 
supplied by the fourth nerve, five times nystagmus, and twice spasmodic 
contraction of the orbicularis were noted. On the whole, palsies and 
other motor disturbances are quite secondary in importance to the 
other ocular phenomena of paresis; are relatively infrequent and in- 
constant. At times slight atactic movements and rarely a well marked 
nystagmus is noted, as witness the cases reported by Deutsch, Hepburn, 
Ballet, and others. These movements are not to be regarded as truly 
nystagmic, but merely as a local incoordination comparable to the 
ataxia and uncertainty of movement noted in the limbs. 

SUMMARY. 

Among the earliest signs of paresis are inequality and irregularity of 
the pupil, and at times impairment of the visual acuity and even 
atrophic changes in the optic nerve. \Ye observe also that the involve- 
ment of the visual apparatus is always gradual and progressive.. Fur- 
ther we note that it is always asymmetrical, or nearly so. 



PAEESIS 489 

In keeping with this fact of asymmetry, we find, as the most frequent 
ocular symptom in paresis, inequality of the pupils. Inasmuch, how- 
ever, as the inequality of the pupils is very frequent in affections other 
than paresis, it is obvious that the association of other phenomena is 
necessary to give to this symptom a specific value. Of itself inequality 
cannot even serve to differentiate between an organic and a functional 
disease. As is well known, it may occur, on the one hand, in tabes, 
paresis, syphilis, or other gross organic disease ; or, on the other, in 
purely functional conditions, such as hysteria and the psychoses. It is, 
therefore, necessary to search for other signs such as impairment or 
loss of the light reaction and anomalies of accommodation and con- 
vergence. In paresis such additional symptoms sooner or later make 
their appearance. 

Irregularity or deformity of the pupils is almost as common as 
inequality. Myosis, on the other hand, is less frequent though it occurs, 
roughly speaking, in about one-third of the cases. 

The usual sequence of the phenomena observed is about as follows: 
at first inequality, irregularity, and impairment or loss of the light 
reflex, consensual or direct, upon one or both sides, with at the same 
time preservation of and persistence for a time of the reaction to ac- 
commodation ; later only do we find impairment and finally loss of the 
reaction to accommodation, the pupil being then inert to all forms of 
excitation. The Argyll-Robertson pupil is, therefore, present during 
a part of the course of paresis. However, we should bear in mind that 
while this is so in many cases, in others the loss of the light reflex may 
be somewhat retarded and the loss of accommodation may, on the other 
hand, occur relatively early, so that the duration of a typical Argyll- 
Robertson pupil in a given case may be short. In this respect paresis 
presents a marked contrast to tabes, in which the Argyll-Robertson 
pupil is a prominent and characteristic feature during almost the 
entire course of the disease. Observers are not wanting who find the 
Argyll-Robertson pupil present in but a small number of paretics. 
Thus, Dawson out of forty cases found it present upon both sides in 
only Hve cases, and on one side in only three cases. Intermediate con- 
ditions also are found in which the light reflex is not wholly lost, but 
in which at the same time the reaction to accommodation is reduced. 
Dawson found this to be the case in 30.76 per cent. Sluggish or slow 
reaction of the pupil to light with diminished movement is not in- 
frequently observed. It appears to constitute a period between the 
normal pupil and the typical Argyll-Robertson pupil. Similarly, the 
accommodation reaction is not infrequently found to be sluggish and 



490 THE EYE AND NERVOUS SYSTEM 

slow instead of being lost. Its occurrence means the beginning of a 
total iridoplegia. Finally we should remember that inequality, irregu- 
larity, deformity, myosis, and mydriasis are either forerunners of or are 
closely associated with anomalies of pupillary action. 

The pupillary changes of paresis are, it need hardly be stated, not 
peculiar to paresis. They are met with in varying degrees in both 
syphilis and tabes, and some of them, such as inequality, are also found 
in purely functional nervous and mental affections, as already stated. 
Not only are all of the pupillary disturbances of paresis observed in 
tabes, but Babinski and Charpentier have shown that the Argyll-Robert- 
son sign is very frequently met with in old cases of syphilis, irrespec- 
tive of signs suggestive either of tabes or paresis. Whether the appear- 
ance of such symptoms justifies a prognosis of tabes or paresis is still 
an open question. That it does so, with or without other symptoms and 
in spite of the presence of the active signs of true nervous syphilis, is an 
opinion to which the author strongly inclines. 

Finally it should be borne in mind that some of the pupillary phe- 
nomena observed in paresis — e.g., inequality and irregularity — may 
vary considerably and may even be intermittent; especially may this 
be noted in the early stages. The light reflex also may in rare instances 
vary ; for example, if greatly impaired though not entirely lost, it may 
be re-established during a remission as in the case observed by Bumke. 
It is even asserted that in very rare cases (Fuerstner, Gaup) the reflex 
to light may be again established after having been entirely lost. On 
the whole, all of the ocular phenomena of paresis are less fixed and 
definite than they are in tabes. Again, irregularity of the pupil, so 
frequently an early sign, is to be looked upon as the beginning of the 
progressive-iridoplegia of which the Argyll-Robertson is a more ad- 
vanced stage and the inert pupils of the terminal period the final 
stage. 

Eyes which are normal, or at any rate in which no symptom can be 
detected, are also met with in paresis, but only in the early stages of the 
affection. Thus, in two cases reported by James, the pupils were equal 
and reacted properly to light and accommodation. In a third case, 
however, while the pupils reacted normally, one was smaller in size 
than the other. In a series of nine cases, studied by Keraval, eye 
symptoms were absent in four cases, an experience which must be con- 
sidered, however, as quite unusual. 

Amblyopia, amaurosis, optic atrophy are all physical signs, the 
detection of which as we have already seen may be of great moment to 
the diagnosis. That amblyopia, transient amaurosis, and even optic 



PAKESIS 491 

atrophy may be the first noticeable physical sign of paresis should be 
especially borne in mind. 

The extra-ocular palsies and anomalies of movement, though not 
striking in paresis, should be remembered especially because of their 
value in differentiating the affection from syphilis, gross organic disease, 
and perhaps tabes. 

The eyes in juvenile paresis present a symptom group similar to 
that found in older subjects. In keeping with the fact that pupillary 
changes are apt to be observed at early ages in paresis, Marchand noted 
sluggish light reaction in a boy of 18 together with inequality of the 
pupils. JofTroy likewise noted Argyll-Robertson pupils, together with 
inequality in a juvenile case. Nonne, in a boy of twelve, noted loss of 
the light reflex in the right pupil and sluggishness of the reflex in the 
left pupil. The pupils were mydriatic and unequal, while the con- 
vergence reflex also was very slight. Raymond, in a girl of twelve, 
noted diminution of the light reflex, likewise with dilated and unequal 
pupils. Regis made a similar observation of a patient of twenty-three. 
There was loss of the light reflex especially upon the left side, together 
with unequal pupils ; there was marked diminution also of the reaction 
of accommodation. In a boy of eighteen, Marchand also noted sluggish 
pupillary reactions, the right pupil being larger than the left. Hirschl 
noted, in a girl of twenty-two, that the right pupil was large and slug- 
gish to light, while the left did not react to accommodation. In a boy 
of sixteen he noted unequal pupils, loss of the light reflex upon both 
sides. In a boy of nineteen he again noted that the right pupil was 
larger than the left and also that there was present a loss of the light 
reflex. In a man of twenty-four, the same observer noted that the right 
pupil was larger than the left, with sluggish reaction to light ; in the 
left pupil the light reaction was lost; both pupils presented sluggish 
Teaction to accommodation. 

In senile paresis, upon the other hand, Toulouse noted inequality of 
pupils in a man of seventy-two ; Marchand, inequality of the pupils in 
a man of sixty, and Doutrebente, inequality of pupils in a man of sixty- 
five, together with sluggishness of the light and consensual reflexes, 
especially upon the left side. 

A subject but little studied and of relatively slight importance is 
the sympathetic pupillary reaction. This consists of a dilatation of 
the pupil which ensues upon irritation of sensory nerves of the skin of 
the face, neck, or other contiguous areas. It is best elicited by pricking 
or pinching the skin. Hirschl found that in all the cases studied by him, 
"the sympathetic reflex was absent when the Argyll-Robertson pupil was 



492 THE EYE AND NERVOUS SYSTEM 

present, except in two. It was also frequently absent when the light reac- 
tion was sluggish, and this was also frequently the case even when the 
light reflex was still preserved. When an Argyll-Robertson pupil was 
present upon the one side and absent upon the other, the sympathetic re- 
flex was absent upon both sides. The loss of the sympathetic reflex, 
however, was also noted by Hirschl in senility and alcoholism, and he 
believes that in cases in which these two factors can be excluded, this 
symptom offers some diagnostic value. Hirschl also observed that in 
cases in which this reflex was absent, there were atrophic changes in the 
sympathetic nerve, sympathetic ganglia, and in the anterior horns of the 
lower and upper dorsal cord. Moeli found that the reaction was much 
more frequently lost in cases in which the light reflex had suffered. 
Hillenberg found similar results. 

Among the pupillary phenomena still to be mentioned is that known 
as the paradoxical pupillary reaction. Here there is an apparent re- 
versal of the light reflex, — that is, an increase of light is followed by 
dilatation of the pupil and diminution of light by contraction of the 
pupil. The occurrence of this reaction is an exceedingly rare one in 
paresis. In fact, but one case, that of Morselli, is known to the 
writer; in this instance dilatation of the pupil followed the light im- 
pression. It is important to bear in mind that a paradoxical pupillary 
light reaction may be apparent only and not genuine, — for instance, a 
weakness or paresis of accommodation occurring at the moment that 
the light test is made, may bring about a dilatation of the pupil. 
Similarly a paresis of the convergence reaction, accompanied it may 
be by divergence of the eyeballs, may also occur at the moment that 
the light test is made, and thus give rise to a dilatation of the pupil. A 
high degree of amblyopia or amaurosis may be associated with an im- 
pairment of accommodation and convergence, and may further account 
for the occurrence of such a phenomenon. Shock, fright, nervousness, or 
hysteria may also play a role. In practice, as far as paresis is con- 
cerned, the paradoxical reaction of the pupil being so rare, may be 
ignored. 

The synergic narrowing of the pupil that accompanies contraction 
of the orbicularis palpebrarum, and which has been in recent years 
studied by A. Westphal and Piltz, and which had been earlier noted by 
von Graefe, by Galassi, and by Moeli, is another pupillary reaction 
which may be mentioned. It has, however, been studied so little in 
paresis, that it cannot be said to possess any symptomatic value. It 
may be added that another pupillary phenomenon, hippus, likewise 
plays no role in the syndrome of paresis. 



TABES 493 

TABES. 

The ocular phenomena of tabes constitute a group of symptoms of 
great diagnostic value. As in paresis, they may involve varied struc- 
tures and functions, — e.g., the iris, its movements and reflexes; the 
various extrinsic muscles, the optic nerve and vision. Tabes is an affec- 
tion with widely diffused lesions and it is but natural that the early 
clinical manifestations should vary somewhat in different cases. Ac- 
cordingly the eye symptoms make their appearance somewhat earlier in 
some cases than in others. 

In general terms, the symptoms of tabes consist of a triad made up 
of incoordination, loss of tendon reflexes, and the Argyll-Robertson pupil, 
— the pupil which no longer reacts to light but in which the associated 
movements of the iris in accommodation and convergence are preserved. 
The presence of this pupil is not infrequently noted as a very early symp- 
tom. It may be noted long before the knee-jerk is lost and before inco- 
ordination makes its appearance. Sometimes it antedates all other symp- 
toms by a number of years. On the other hand, its appearance may be 
much delayed and in rare cases it may not be observed at all even after 
other symptoms of tabes have existed for many years. While present 
in the great majority of cases of tabes, it cannot therefore be claimed 
as a necessary symptom. 

It is important in this connection to bear in mind that the loss of 
the light reflex is a symptom which in all probability makes its appear- 
ance gradually. In examining the eye, a difference in the promptness 
and general character of the light reaction and the reaction to accommo- 
dation should always be noted. A striking contrast between the two 
reactions, such as a slight, slow or sluggish light reflex and a prompt 
reaction to accommodation possesses an unmistakable significance. As 
a matter of fact there are many cases in which, in the incipient period, a 
fully developed Argyll-Robertson pupil is not present and in which a 
sluggish or other change in the light reaction is the only symptom noted. 
In a few cases also it has been observed that the Argyll-Robertson pupil 
is not definitely or permanently established and exists only as an inter- 
mittent symptom. Thus Eichhorst reports two cases of intermittent 
Argyll-Robertson pupils, both occurring in women. Treupel reports 
another occurring in a man. Camus and Chiray report a most interest- 
ing case in which the Argyll-Robertson pupil was present, accompanied 
by gastric crises. It occurred only during the crises and lasted only as 
long as the crises. 

In estimating the frequency of the Argyll-Robertson pupil in tabes, 



494 THE EYE AOT) KEKVOUS SYSTEM 

several factors should be considered. First, while it is true that this 
pupil is frequently an early symptom, it is not rarely a late symptom ; 
therefore cases of the fully developed disease would, other things equal, 
present the symptoms in a larger proportion. Secondly, the proportion 
varies according as the observer excludes or includes pupils in which 
the light reflex has not been entirely or absolutely lost but in which it 
has been unmistakably impaired. It is not surprising therefore that 
there should be some differences in the results of various observers and 
yet these differences are not as great as one would suppose. Dillmann, 
for instance, found loss of the light, reflex in 76 per cent, of cases in 
the fully developed stage of the disease. In the early stage he found 
only 23.7 per cent. Leimbach found- the symptom in 75.6 per cent, in 
the fully developed disease and 47 per cent, in the early stage. The 
chief variation is found in the determination of the symptom in the 
early stage and here the element of the personal judgment — the per- 
sonal equation of the observer — doubtless plays a role. On the other 
hand it is difficult to reconcile the findings of other observers such as 
Mann, who found the symptom in 79 per cent, of early cases, — i.e., cases 
in which no pronounced ataxia had yet shown itself. Statistical studies 
have been made by a large number of observers. Uhthoff, for instance, 
in three hundred cases noted rigidity of the pupil in 71 per cent. ; ex- 
cluding cases in which a slight reaction of the pupil was still preserved, 
the percentage became 62. In averaging the results of other observers, 
for instance, Gowers, Bernhardt, Voigt, Thomsen, Siemerling, Vincent, 
Dillmann, Berger, Mobius, Marina, Forster and Mann, Uhthoff ob- 
tained a percentage of 67, which nearly corresponded to his own results. 
Among other studies there should be mentioned the following : Harris, 
82.6 per cent. ; Leimbach, 84 per cent. ; Bramwell, 8.6.1 per cent., and 
also 72.2 per cent. ; Mott, 80 per cent. ; H. M. Thomas, 63.6 per cent. ; 
Thoenes, 75 per cent ; Collins, 80 per cent. ; Fulton, 67 per cent. The 
writer found among thirty rather advanced cases in the wards of the 
Philadelphia Hospital 63.3 per cent. These figures do not, it will be 
admitted, vary very greatly. However, such results as were obtained by 
F. S. Pearce, — namely, one hundred and fifty cases of Argyll-Eobertson 
pupil in one hundred and fifty-six cases of tabes, that is, 96 per cent., 
— are difficult to explain. The percentage found by Riley, who found 
Argyll-Robertson pupil in only twenty-eight cases out of sixty-one, — 
namely, 46 per cent., — must impress one as unusually small. This is 
also true of Vaughan's results, who found pupillary rigidity in only 40 
per cent. 

Very interesting and important are the results obtained by Rochon- 



TABES 495 

Duvigneaud and Jean Heitz, inasmuch as their studies were made by a 
special method. They examined seventy-seven patients who for the most 
part were from the service of Professor Dejerine. The patients belonged 
to all of the periods and the most diverse forms of the disease. The 
examinations of the pupillary reactions were made in an entirely dark 
chamber under special precautions in order to determine as far as pos- 
sible the exact condition of the light reaction. A gas-lamp furnished the 
illumination. The patient was separated from the latter by an opaque 
screen. After lowering the gas, without absolutely extinguishing it, the 
eye to be examined was placed in the most complete darkness for several 
minutes. Then the operator suddenly raised the gas flame and the light 
was projected on the eye by means of an ophthalmoscopic mirror. The 
eye not to be examined remained covered. 

Of forty-eight patients examined by these observers, twenty-three 
had lost all trace of light reflex, fifteen presented a slight reaction to 
light, and two reacted almost normally but not entirely so. From this it 
appears that in 47.9 per cent, the light reflex was lost entirely and that 
in about 35 per cent, it was lost only incompletely; that is, a total of 
about 83 per cent, presented lost or impaired light reflex, which accords 
fairly well with the results of other observers. The observations of these 
writers would go to show that the methods pursued by them reveal a 
partial preservation of the light reflex when to ordinary tests such 
preservation would escape notice. Thus they examined twenty-nine 
cases by the ordinary methods, — I.e., with partial obscurity and moving 
light. Of these only four presented an incomplete loss of the light reflex, 
a percentage of 13, as compared with 35. The authors also lay especial 
stress on the importance of studying the dilatation of the pupil in the 
dark. This they maintain is an active act, an impairment of which is 
of profound significance. 

Statistical studies for reasons already pointed out can hardly be 
expected to yield precise results. The conclusion, however, is unques- 
tionably justified, and this indeed is the sum total of individual clinical 
experience — that the Argyll-Robertson pupil is present in a large ma- 
jority of cases. 

An unilateral Argyll-Robertson pupil is relatively infrequent. Thus, 
Berger found an unilateral Argyll-Robertson pupil only three times in 
one hundred and nine tabetics, and Gowers once in seventy-two. H. M. 
Thomas, on the other hand, states that he found the Argyll-Robertson 
pupil in seventy out of one hundred and eleven cases and upon both 
sides in fifty-seven; that is, it was unilateral in thirteen. Probably 
pupils with but slightly impaired reaction were here excluded. Out of 



496 THE EYE AND NERVOUS SYSTEM 

thirty of the writer's cases, it was unilateral in four. It remains an 
unquestionable fact that in tabes an unilateral Argyll-Robertson pupil 
is the exception, and there is in this respect a decided departure from 
the symptomatology of paresis, in which affection this symptom is 
observed much more frequently. 

Einally it should be stated that the Argyll-Robertson pupil may be 
found when the pupil is of average or normal size, when it is myotic or 
when it is dilated. 

Irregularity or deformity of the pupil is found quite commonly in 
tabes, as in paresis and syphilis. Joffroy states that he has never seen 
a case of Argyll-Robertson pupil without some deformity in the latter. 
This statement, while rather sweeping, accords in the main with the 
experience of other observers. Deformity is at times the initial pupillary 
symptom observed and it may be regarded as a precursor of the changes 
in the pupillary reflexes later to supervene. Joffroy, indeed, regards it as 
of the same semeiologic value as the Argyll-Robertson pupil. Bumke also 
insists upon the importance of the loss of the circular shape of the pupil. 

Next to the Argyll-Robertson pupil in importance, is the size of the 
pupil. The pupil may be normal, unusually small, or dilated. Very 
frequently it is decidedly contracted and this diminution in size may 
be moderate in degree or excessive. Pin-point pupils are occasionally 
met with. Myosis frequently accompanies the Argyll-Robertson pupil, 
though it is by no means a necessary concomitant, for the latter may be 
associated with pupils of normal size or pupils in a state of mydriasis. 
Thus, Erb found it in 44 per cent. ; Uhthoff, Dillmann, and Voigt found 
it in a somewhat smaller number. Their findings averaged by Bumke 
amount to about 24 per cent. Collins found the pupils small in 45 per 
cent; the writer in 36.6 per cent. Rochon-Duvigneaud and Heitz found 
40 per cent, of bilateral myosis and 9 per cent, of unilateral myosis. 
In every case of myosis there was a pure Argyll-Robertson pupil, ex- 
cept in three. In the latter contraction to convergence was slow and 
incomplete. External ophthalmoplegias were not found associated with 
the myosis. These authors also point out that optic atrophy is more 
frequently found associated with mydriatic eyes than with myotic eyes. 
In forty myotic patients, they found only three with total atrophy 
and blindness and three with beginning atrophy. 

Myosis, it would appear, is accompanied quite regularly by the 
Argyll-Robertson sign. In a number of cases, however, a slight reaction 
to light is preserved. It is very exceptional, further, to find a myotic 
patient with optic atrophy and more rarely still with internal or ex- 
ternal ophthalmoplegia. 






TABES 497 

From an analysis of eleven hundred cases, Dufonr comes to the 
conclusion that myosis, associated with other disturbances of the pupils 
or of the nervous system, confirms the diagnosis of syphilis or organic 
nervous disease. . 

Mydriasis is met with much less frequently than myosis. Thus 
Riley in sixty-one cases found myosis in twenty-eight and mydriasis in 
eight. Bramwell in one hundred and fifty-five cases of tabes found the 
pupils contracted in sixty-nine and dilated in eight. In twenty-seven 
they were of medium size. Collins found mydriasis in only 5 per cent, 
and when found it was nearly always associated with irregularity of the 
pupils. Rochon-Duvigneaud and Heitz found mydriasis in 20 per cent, 
bilateral and 11 per cent, unilateral of their seventy-seven cases. My- 
driasis was accompanied by a complicated Argyll-Robertson pupil, — that 
is to say, a pupil in which the reaction to accommodation was also im- 
paired. These writers point out that when blindness is present, it is as 
a rule, accompanied by mydriasis. They found seven blind tabetics 
with bilateral mydriasis and two with unilateral mydriasis. In all of 
the cases the reflex to convergence was absent or diminished. In twelve 
cases of mydriasis, they found two with bilateral internal ophthalmo- 
plegia, ten with unilateral ophthalmoplegia, and in two cases there was 
a nuclear palsy of the third pair, incomplete for the external muscles 
but complete for the ciliary muscles. In the remaining cases, ten in 
number, all unilateral, there were five times found besides the internal 
ophthalmoplegia, also various palsies of the external muscles of the third 
nerve supply; especially was ptosis found. Once internal ophthalmo- 
plegia with palsy of the sixth nerve was found ; four times internal 
ophthalmoplegia only was present. In five cases of mydriasis there was 
no blindness or paralysis of accommodation. 

The mydriatic pupil is accompanied by various other disturbances, 
a fact which is directly opposed to the findings in the myotic pupil. 
Thus the light reflex is totally lost, the convergence reflex is always in- 
complete or lost, while optic atrophy and ophthalmoplegias are fre- 
quent, Finally it is mostly in cases with mydriasis that inequality 
of the pupils is found. 

There is a group of tabetic pupils in which the sphincter iridis pre- 
sents a reflex paralysis, while the ciliary muscle remains intact and 
the accommodation is preserved. This paralysis of the sphincter could 
perhaps be attributed to a limited nuclear lesion, for the nucleus of 
the circular fibres of the iris is entirely distinct from that of the ciliary 
muscle. Again, while paralysis of accommodation is often accompanied 
by paralysis of the external muscles, in not one case did Rochon-Duvig- 
32 



498 THE EYE AND NERVOUS SYSTEM 

neaud and Heitz find such a paralysis associated with the isolated palsy 
of the sphincter iridis. 

It has been observed that during a gastric crisis, pupils previously 
noted as normal may dilate, but that in such case they return to their 
former size after the crisis has subsided. Such an observation has been 
made by Rochon-Duvigneaud and Heitz, dilatation and subsequent re- 
turn to normal occurring in one of their patients at every gastric crisis. 
Similar observations have been made by Trousseau and Raymond. 

Schmeichler states that he has never seen unusually large pupils in 
the early stages of tabes. This is an important observation and in keep- 
ing with general experience. 

It is interesting to note also that atropine gives an incomplete or 
imperfect dilatation of myotic pupils, and that pilocarpine in mydriasis 
yields a contraction which is not very pronounced. 

Mydriasis, according to Rochon-Duvigneaud and Heitz, always ac- 
companies the complicated Argyll-Robertson pupil, — that is, the pupil 
in which the light reaction is totally abolished, but in which the reac- 
tion to convergence is also lost or is poor. In a certain number of cases, 
mydriasis depends upon blindness. When the mydriasis is accompanied 
by a preservation of vision the state of the accommodation should be 
investigated, taking into account, of course, the age and refraction of 
the patient. Usually under these circumstances the accommodation is 
paralyzed. There is then a total internal ophthalmoplegia, involving the 
sphincter iridis and the ciliary muscle. Very rarely temporary dila- 
tation of the pupils, that is " alternating mydriasis," is met with. It 
is much less frequent in tabes than in general paralysis. 

Inequality of the pupils should, even if slight, be regarded with sus- 
picion, especially if one of the pupils reacts sluggishly to light or shows 
impairment of the light reflex, no matter how slight. Collins noted 
inequality in 23 per cent, of his cases. He adds that it was probably 
not noted in cases in which the inequality was very slight. Bramwell 
found the pupils unequal in 34.8 per cent. Yaughan found 60 per cent, 
of inequality of the pupils, a percentage which it cannot be denied is 
out of keeping with general experience. The writer found 46.6 per 
cent, of decided inequality and 13.3 per cent, of very slight inequality. 
It will be noted that the percentage of inequality in tabes is decidedly 
less than in paresis. Among other statistics are those grouped by 
Bumke, — namely, Bernhardt, who saw inequality of the pupils in 
43 per cent., Yoigt in 40 per cent., Dillmann in 34 per cent., Forster 
in 33 per cent., Uhthoff in 28 per cent., and Berger in 27 per cent. 
Inequality, if observed as an isolated sign and without other ocular 



TABES 499 

or nervous symptoms, lias no special significance. In other words, in- 
equality may be perfectly physiological. It must, however, be borne in 
mind that a physiological inequality is usually slight. (See Chapter 
VI, p. 314). 

The optic nerve is frequently involved in tabes. There is present in 
such cases a simple and progressive atrophy of both optic nerves, — an 
atrophy which may and frequently does lead to complete blindness. It 
is a symptom which is frequently associated with an Argyll-Robertson 
pupil, but it may exist without the latter and it may be the first and 
only symptom. Indeed, simple optic atrophy, in the absence of other 
signs, is very suggestive of tabes. Usually it makes its appearance early ; 
it is only in exceptional cases that optic atrophy is a late symptom. Col- 
lins believes that the discoloration of the optic nerve begins within five 
years after the onset of the disease. He regards it as an early symptom 
and believes that the changes probably start peripherally in the retina. 
So frequently is early optic atrophy noted that it is justly counted along 
with the Argyll-Robertson pupil as one of the first symptoms of tabes. 
Mott also regards limitation of the visual field and optic atrophy as the 
early signs of tabes. Gowers has seen optic atrophy fifteen or twenty 
years before other tabetic signs made their appearance. Not infre- 
quently one eye is involved earlier and to a larger extent than its fel- 
low, but the optic atrophy is never really unilateral. Defective vision 
is the eventual result. The first indication is a narrowing of the visual 
field. The impairment usually consists of an irregular concentric con- 
traction ; at times the field is irregularly indented. There may be lit- 
tle or no diminution of the acuity of the central vision and no visible 
pallor of the disc. Central scotomata are very exceptional ; when pres- 
ent they are frequently explicable on the ground of a complicating alco- 
holism. Occasionally the visual field is limited on one side, as, for 
instance, in the case reported by Halbau, in which the visual field in 
the right eye, especially for red, was limited on the temporal side. 
Hemianopsia, incomplete in character, is a rare anomaly of the field and 
is apparently due to an asymmetrical degeneration. It has been noted 
by Gowers, Berger and Gabrielides. In Gowers's and Gabrielides's 
cases, there was a double temporal hemianopsia referable to a change 
in the chiasm. In Berger's case, there was a right homonymous hemian- 
opsia. There were also present Argyll-Robertson pupil, double optic 
atrophy, palsy of the right fourth nerve and other symptoms of tabes. 
The loss may also involve early the color perception, the vision for green, 
red, and blue being affected in the order named. Dyschromatopsia was 
first noted in tabes by Benedict. Generally speaking dyschromatopsia 



500 THE EYE AND NERVOUS SYSTEM 

is partial and concerns mostly red. and green, the red being taken for 
black and the green for gray. A curious symptom occasionally noted in 
tabes is green vision. Such a case, for instance, has been placed upon 
record by W. Dodd, in which the patient saw everything a bright emerald 
green in color. Occasionally he saw rose-colored spots in the green. 
These colors increased in intensity when he was fatigued. He oc- 
casionally also saw a brilliant light. In 1891 diplopia had been noted 
from which the patient recovered. Ocular vision remained good until 
1897. Examined in September, 1898, green vision was noted. The 
vision of the right eye was 2-60, of the left 1-60. 

Rarely optic atrophy is accompanied by photophobia and blepharo- 
spasm. 

Statistics as to the relative frequency of optic atrophy in tabes vary 
very widely and are on the whole unsatisfactory. Collins found optic 
atrophy in 14 per cent., Bonar 8 per cent., Thomas 10 per cent., Riley 
11.4 per cent., Bramwell 22 per cent., Sarbo 30.4 per cent. Eulton 
found only 2 per cent., Vaughan noted 50 per cent., Dillmann 42 per 
cent., Berger 44 per cent., Leimbach 16.75 per cent, and Grosz in 
88 per cent. Mott places the percentage of cases of optic atrophy in 
tabes at 80. Pearce, on the other hand, found only thirty cases of 
optic atrophy in one hundred and nineteen cases. Marina observed 
optic atrophy in 9 per cent., Grosz in 88 per cent., Uhthoff in 20 per 
cent, Bernhardt in 10 per cent., Berger in 44 per cent., and Silex 
15 per cent Oliver believes that optic atrophy is met with in 10 to 
15 per cent. 

It has long been held that when tabes begins with optic atrophy 
that the affection soon becomes arrested. The very exhaustive investiga- 
tions, however, of Andre Leri fail to substantiate this position. Numer- 
ous observations fail to prove that the occurrence of optic atrophy is 
attended by a cessation of the progress of the disease. A case reported 
by Bramwell will serve as an illustration. In a man of thirty, two 
years previous to all signs of tabes, optic atrophy with blindness and 
dilatation of the pupils were observed. Shortly after, however, other 
important signs of tabes made their appearance. Collins also noted that 
in no case did the superimposition of blindness seem to have any effect 
upon the course of the disease. Gowers maintains that optic atrophy 
is generally an early symptom beginning before incoordination is de- 
veloped and that in a large number of cases ataxia does not supervene. 
Benedict believes that tabetic motor symptoms, even after they have 
attained a high degree, retrograde after the optic atrophy appears. In 
eight out of thirty-three cases observed by Bramwell, the developmenl 



TABES 501 

of the optic atrophy seemed to be attended by an amelioration of the 
symptoms and a retardation of the tabetic process. In twelve cases, how- 
ever, the development of the optic atrophy was not followed by an 
amelioration of the symptoms and had no retarding effect upon the 
course of the disease. In nine out of twenty-six cases in which optic 
atrophy was very marked, the development of the optic atrophy coin- 
cided with a rather apparent arrest in the tabetic process. 

The details obtained by some observers are interesting; thus Bram- 
well, who studied one hundred and fifty-five cases of tabes, noted that 
in seven of his cases, in which optic atrophy was present, the symptom 
was much more marked in one eye than in the other and that in three 
cases it was limited to one eye. In seven cases it was slight, in seven 
marked, and in twenty-one very marked or complete, the patient being 
almost blind, quite blind, or only able to distinguish light from dark- 
ness. In one case, double optic neuritis, marked in the left eye and 
slight in the right, preceded the optic atrophy. Such an occurrence 
suggests, however, an active syphilitic process rather than tabes. In 
nine patients dimness of vision, due to optic atrophy, w T as the initial 
symptom of the disease. In twenty-five of the cases in which optic 
atrophy was present, there was ataxia. 

Ocular palsies are extremely common in tabes and in quite a large 
number of cases they occur as very early symptoms. They involve 
variously the third, fourth and sixth nerves. They usually appear sud- 
denly and may disappear with or without treatment. Their duration is 
usually short, lasting a few w T eeks, a few days, or indeed only a few 
hours. They may, how T ever, be permanent, though this is rare and 
points to a syphilitic rather than to a tabetic origin. However, a per- 
sistent ocular palsy may be present in the very beginning of the affec- 
tion but is more frequently observed in the later stages of the disease. 

The ocular palsies of tabes present nothing that is characteristic. 
Though they develop rapidly, they usually disappear gradually and 
sometimes slowly. The muscular involvement is seldom complete and 
is usually limited to one eye. Further, the palsy is never a palsy of 
associated muscles or movements, but always involves, irregularly, dif- 
ferent muscles of one or both eyes. Occasionally a complete ophthalmo- 
plegia appears. This again is to be regarded as pointing to a syphilitic 
rather than a tabetic origin. It is noteworthy that in syphilitic ocular 
palsy, a large number of muscles are affected at the same time while 
in tabes involvement of a single muscle is the rule. It is further in- 
teresting and in practice important to note that it is extremely rave in 
tabes to find ocular palsies without a loss of the light reaction. Opin- 



502 THE EYE AND NERVOUS SYSTEM 

ions differ as to the muscles most frequently involved. Thus Swanzy 
and others believe that the external rectus is most frequently affected, 
while Charcot believed that the oculomotor nerve supply suffered first. 
Javal found that the levator was most frequently involved, then the in- 
ternus, the externus and the superior oblique. Certain it is that ptosis 
is very frequent. It may be slight or pronounced and may be single or 
double. It does not always depend upon paralysis, especially when 
slight; it is then due to atony of the levator and under such circum- 
stances may be inconstant. Finally it rarely exists alone. Very fre- 
quently other muscles are involved, giving rise to a diplopia. This 
diplopia is usually due to a palsy in one eye, is very common, and is 
usually transitory. Transitory diplopia is frequently an important 
point in the history of the early period of a case of tabes. At times the 
diplopia depends upon palsy of the external rectus, at times it depends 
upon palsy of the internus or other muscles of the oculomotor supply, — 
e.g., the superior rectus. Involvement of the fourth nerve, — especially 
isolated involvement, — is infrequent, if not rare. The diplopia, though 
transitory for the most part, is in rare cases permanent. Further, hav- 
ing once disappeared, it may recur. It is sometimes an exceedingly 
early symptom; thus, in a case reported by Harris, it existed for six 
years before other symptoms of tabes made their appearance. 

Rarely muscles other than the ocular muscles may be palsied at the 
same time. Thus in a case reported by Turner, a man of forty, there 
was some ptosis and also some bilateral palsy of the soft palate and 
paralysis of the vocal cords. Rarely also instead of an ocular palsy, a 
blepharospasm may be noted or a blepharospasm may precede the de- 
velopment of ocular palsies, 

A most interesting form of ocular palsy has been reported by Egger. 
In one of his clinics, Dejerine presented a case of bulbar tabes in which 
of the twelve pairs of cranial nerves, the affection had only spared the 
IV pair, the III, V, VI, VII, VIII, IX, X, XI, and XII pairs were all 
more or less strongly involved. A bilateral and absolute deafness char- 
acterized the lesion of the VIII pair, whilst a slight ptosis of the left 
upper eyelid and a paresis of the left internal rectus were the sole 
manifestations of involvement of the III pair. Although there was no 
paralysis of any one muscle of the eye, there was notwithstanding a 
marked reduction in the motility of the eyes. When in the normal 
individual the head is turned to one side — for example, to the left 
on its vertical axis — the eyes in order to preserve their primary po- 
sition move slowly in the opposite direction — i.e., to the right — 
and compensate by movement equivalent to the degree of rotation 



TABES 503 

effected by the head, but at a given moment the eyes abandon this 
primary position and a return movement ensues. This go and 
come repeats itself about a dozen times for one complete rotation of 
the body around its vertical axis and gives, if the rotatory movements 
acquire a certain speed, the aspect of a nystagmus which has been called 
the transverse nystagmus of rotation. These movements are regulated 
by the pressure of the liquid in the semicircular canals, varying for 
each position of the head. Each canal is sensitive for the movements 
affecting its plane. A rotation in a horizontal plane to the left, how- 
ever, affects only the left horizontal canal, in the interior of which the 
liquid, thanks to the law of inertia of matter, concentrates toward the 
ampulla and there produces an excitation which informs the centre as 
to the direction of the change of position. The movements in the sagittal 
or vertical plane are only registered by the sagittal or superior-semi- 
circular canals and the movements in the frontal and transverse planes 
by their respective canals, and as each canal perceives only one movement 
— that is to say, the change of position in its own plane — it can only ex- 
cite a compensatory movement which the direction of the change of posi- 
tion calls forth. It is thus that the horizontal canals preside over the com- 
pensations of lateral movements in innervating the external and internal 
recti. The sagittal canals regulate the vertical oscillations and the 
frontal canals the muscular apparatus affecting compensatory rotation 
around the visual axis. If the tips of the middle and ring fingers of 
each hand are lightly placed against the eyeballs in the healthy man, 
these movements can readily be felt. In Egger's case not the least trace 
of movement was transmitted to the fingers for any change of position. 
The phenomena of nystagmus of rotation and of post-rotatory nystagmus 
were lost. In semidarkness, where the retinal reflex did not impede by 
the fixation of objects the influence of the labyrinth, he observed in the 
two open eyes that the latter, instead of making a movement opposite to 
that of the direction of the head in order to preserve their primary posi- 
tion, remained absolutely immobile in their orbits. They did not par- 
ticipate in the movements of the head. This absence of compensatory 
movement is the rule with deaf-mutes and is observed in some cases of 
internal otitis. Among tabetics it had up to the case reported by Egger 
not been observed. The name labyrinthic ophthalmoplegia has been 
proposed by Egger for this symptom. 

The cause of the diplopia or ptosis of tabes appears, according to 
Harris, to be some vascular derangement or slight ependymitis in the 
nuclear region. Pierret believes that it begins as a nuclear affection and 
extends gradually downward toward the periphery as a descending 



504 THE EYE AND NERVOUS SYSTEM 

neuritis and here continues after the nuclear involvement has disap- 
peared. Dejerine says that not all ocular palsies in tabes are of nu- 
clear origin, and Swanzy claims that a complete and permanent paralysis 
of an isolated muscle is due to a peripheral lesion and not nuclear. 
Complete oculomotor palsy, rarely noted as an early symptom, is 
probably always due in part at least to an involvement of the oculo- 
motor nucleus. This was the finding in a case of Marina in which a 
third-nerve palsy disclosed at autopsy a degeneration of the oculomotor 
nucleus besides a neuritis of the trunk. It is probable also that in cases 
where an ocular palsy becomes permanent, there is also involvement 
of the nucleus. 

Some idea of the frequency of ocular palsies may be gained from 
the following figures : Thus, H. M. Thomas found affections of the ex- 
ternal muscles in thirty-three out of one hundred and eleven cases. 
G. Oram Ring states that palsies of the ocular muscles occur in from 
twenty to twenty-five per cent., and that they are usually found in the 
preataxic stage and not infrequently exist as initial symptoms. Harris 
noted diplopia and other ocular palsies in 11.3 per cent, of his cases. 
Diplopia due to paralysis of the external rectus of one side, Riley found 
in fourteen of sixty-one cases. Bramwell noted some form of ocular 
palsy in 16.1 per cent., ptosis in 7 per cent. In seven of his cases, the 
ptosis was unilateral, in four bilateral. Paralysis of the external rectus 
occurred in 6.4 per cent., unilateral in six, bilateral in four. More ex- 
tensive paralysis of the external muscles occurred in 2.5 per cent. Ley- 
den and Goldscheider found palsy of external ocular muscles in from 
40 to 50 per cent, of their cases. (See Chapter Y, p. 209). 

Next, perhaps, in clinical importance to the phenomena thus far 
considered are loss or impairment of the reactions of the pupil to accom- 
modation and to convergence. Loss or impairment of these functions 
may exist together with loss of the light reflex, though such an asso- 
ciation is infrequent. Further, when accommodation and convergence 
are lost, it is usually in connection with a large and dilated pupil. 
Ophthalmoplegia interna is very rare in tabes and, as may be inferred, 
is indicative of an active syphilitic process rather than tabes. UhthofT, 
however, found ophthalmoplegia interna in 5 per cent, of his cases and, 
it should be added, always unilateral. The latter fact alone suggests 
the special character of the lesion at work, a lesion not common to 
tabetics as a whole. 

It appears that paralysis of convergence is somewhat more rare than 
paralysis of accommodation. Thus of thirty cases studied by the writer, 
paralysis of accommodation was found twice, though six cases revealed 



TABES 505 

an impairment of accommodation. The convergence reaction was absent 
but once and impaired but twice. Similar results were obtained by 
Rochon-Duvigneaud and Heitz. These investigators found only three 
cases with loss of convergence in seventy-seven. Collins again found in 
one hundred and forty cases loss of accommodation in nine. 

Paradoxical pupil is an exceedingly rare symptom in tabes, if in- 
deed it ever really occurs. In such a pupil, dilatation follows exposure 
to light, while contraction ensues upon darkness. This phenomenon is 
open, however, to gross misinterpretation. Very frequently the dilata- 
tion observed is in reality due to an impairment of accommodation, or 
more rarely to a weakness of the convergence reaction. The sphinc- 
ter of the iris being weak makes, perhaps, an ineffectual attempt to shut 
down and then suddenly gives way and dilates. In the only case ever 
observed by the writer, involvement of accommodation and convergence 
was clearly present. Piltz (cited by Bumke) reports a case of double 
optic atrophy (tabetic?), in which upon sudden shading of one eye, 
both pupils became distinctly narrower. Upon sudden illumination, the 
left pupil did not react at all, while the right contracted slightly, both 
directly and consensually. It would appear that the literature fails to 
offer a case entirely free from objection. (See also page 493.) 

Hippus, though very rare in paresis, has been noted in tabes, as, for 
example, in the case noted by Hartman. The case was that of a girl of 
twenty. The pupils were otherwise normal and with normal reactions. 
There was present, however, a high degree of optic atrophy with marked 
impairment of vision and concentric contraction of the visual fields. 

Atactic movements of the eyeballs are not infrequently observed, 
though they rarely approximate a true nystagmus. It is probable that 
this symptom when present in the lesser degree is expressive merely of 
the general factor of incoordination, though when true nystagmus is 
present a special cause seems indicated. Harris suggests that it is 
probably due to nuclear disease. Bramwell noted jerking on extreme 
lateral deviation in 2.5 per cent, of his cases. Fulton noted it in 2 per 
cent, of his cases. In two out of thirty cases examined by the writer 
nystagmus was present ; in two atactic movements, moderate in degree, 
and in eleven very faint atactic movements were noted. 

Pain in or about the eyeball is sometimes observed, especially early 
in the history. It not infrequently assumes the form of a periorbital 
neuralgia. Such a neuralgia may prove a precursor of optic atrophy, as 
in a case observed by Galezowski. Areas of anaesthesia may be found 
also in the course of the fifth nerve. Very rarely the pain assumes the 
form of ocular crises, as in the case reported by Pel. In the latter the 



506 THE EYE AND NERVOUS SYSTEM 

patient suddenly experienced violent burning and knife-like pain in 
and about both eyes. There were present also cramp-like contractions 
of both orbiculares, intense lachrymation and redness and injection 
of the conjunctiva?. The neighboring structures were hypergesthetic. 
The crises lasted from two to three hours. They were attributed by 
Pel to a neuritis of the ciliary nerves. They call to mind very forcibly 
the attacks termed ophthalmic migraine every now and then noted in 
paresis. (See Chapter XVII.) 

Lachrymation is not infrequently observed in tabes and is regarded 
by some writers as an early symptom. It is not infrequently associated 
with myosis. Bramwell found it in 1.2 per cent, of his cases. 

Other pupillary phenomena than those mentioned above may be ob- 
served in tabetics, but they have very little clinical value. Such, for 
instance, is the psychic pupillary reflex of Piltz which is independent 
of the presence of a direct light, the patient's pupil reacting or con- 
tracting in accordance to his mental conception of the presence of a 
bright light or darkness. Similarly in blind tabetics, one can oc- 
casionally secure movements of the pupil by asking the patient to fasten 
his mind upon some object supposed to be in the distance and then 
again upon some object which is close at hand. In such case a psychic 
reaction to accommodation may be observed. The writer has demon- 
strated such a reflex in the case of a blind tabetic sailor, who, when asked 
to think of a ship barely discernible upon the horizon, would have a dila- 
tation of both pupils, and when asked to think of reading a newspaper 
in his hand, the pupils would distinctly contract. The synergic pupillary 
reaction of Piltz and Westphal may also be observed in tabetics, though 
it has no special clinical value. Piltz found that the pupil contracted 
synergically with the orbicularis palpebrarum in 43 per cent, of tabet- 
ics. The writer found it present in only 3 per cent, of his cases and 
in most of these it was slight and not pronounced. In one of the 
writer's cases the pupil, instead of contracting, distinctly dilated. 

For the most part also, the reflex dilatation of the pupils to sensory 
irritation is found to be absent in tabetics. It was actually present in 
but two of the writer's cases; faint dilatation was noted in a slightly 
larger number. 

SUMMARY. 

The ocular findings of tabes consist briefly of the Argyll-Robertson 
pupil, of atrophy of the optic nerve and of ocular palsies, the latter 
being usually fugacious and rarely persistent. Any or all of these 
symptoms may make their appearance early and not infrequently ante- 



TABES 507 

date the general symptoms of tabes. Impairment of the light reflex 
and beginning optic atrophy vie with each other as to the frequency 
of their early appearance. Ocular palsies also, as we have seen, may 
make their appearance at an early stage. Again, irregularity of the 
pupils may antedate a distinct impairment of the light reflex and its 
occurrence should always be regarded with suspicion. The same is 
true of inequality, though this is not observed as often in tabes as in 
paresis. 

Myosis, other things equal, is also an early symptom ; mydriasis, on 
the other hand, is a relatively late symptom and is frequently associated 
with advanced optic nerve atrophy and blindness. At times it is also 
associated with loss of the reaction to accommodation and of the reaction 
to convergence ; ophthalmoplegias also are frequently found associated 
with a mydriatic pupil. Finally, loss of the accommodation and con- 
vergence reactions, if present, are usually observed in a rather advanced 
stage of the disease. An initial ophthalmoplegia at an early stage, it 
will be remembered, speaks rather for a syphilitic lesion than for 
tabes. 

The eye symptoms of juvenile tabes on the whole resemble those of 
the disease in the adult. An analysis of twenty-four cases, reported by 
various authors, shows that an Argyll-Robertson pupil and beginning 
optic atrophy are among the early symptoms. Isolated muscle palsies, 
strabismus and ptosis are also present. In a case reported by Bloch 
there was inequality of the pupils, the left being myotic. The right 
pupil did not respond either to light or accommodation, the left reacted 
to light. In a case reported by Janus, a woman of twenty, the upper 
eyelids were slightly retracted, the left more than the right, the pupils 
unequal, the right larger than the left, both Argyll-Robertson and com- 
plete paralysis of the external rectus. In another case, a boy of sev- 
enteen, there were gross choroido-retinal changes in the right eye, to- 
gether with vitreous opacities. The right pupil was sluggish to direct light 
but active consensually. In another case, a girl of fifteen, the changes 
were limited to unequal pupils, the right being larger than the left; 
both reacted feebly to light. In four out of six cases in infantile tabes, 
collected by Idelsohn, the Argyll-Robertson pupil was present. In four 
cases of juvenile tabes, reported by Maas, there was in one gray optic 
atrophy and Argyll-Robertson pupils in both eyes. In another unequal 
pupils, loss of the reflex to light and convergence in both eyes and also 
choroido-retinitis. In two others there were present unequal pupils, 
gray atrophy and Argyll-Robertson pupils upon both sides. In one of 
these there was a coloboma of the left eye. 



508 THE EYE AND NERVOUS SYSTEM 

FUNCTIONAL INSANITIES. 

In the insanities, — that is, the functional psychoses (excluding, of 
course, under this head paresis, tabes with psychoses and organic brain 
disease generally), — the ocular symptoms presented are relatively unim- 
portant and possess but little clinical value. There are no pupillary 
changes which are peculiar to any mental disease. All efforts that have 
aimed to bring special ocular symptoms in accord with individual in- 
sanities have failed. Notwithstanding it is of importance to review 
briefly some of the studies that have been made in this field and to con- 
sider briefly the inference that it is possible to draw from them. 

The changes that have been studied relate mainly to the pupil. 

According to Dagonet, disturbances of the motility of the iris and 
inequality of the pupils may be found in both normal and insane in- 
dividuals. In the latter it is in the chronic forms that anomalies are 
more frequently observed. In mania a dilatation was observed by 
Dagonet, a dilatation which may alternate with exaggerated contrac- 
tion of the pupils. Minck found spasmodic myosis in periodic mania 
and in the puerperal psychoses, and mydriasis in acute mania. Regis 
writes, pupillary inequality with variable light and accommodation re- 
flexes is often observed in psychoses due to autointoxications, but this 
inequality is very changeable from one day to another and often on the 
same day. Stephani and Morpujo see a relation between the degree of 
myosis and development of the psychic disturbances. Thomsen says that 
pupillary immobility exists in 2.1 per cent, of the insane, exclusive, of 
course, of paresis. He found it once among one hundred and eighty- 
nine epileptics, in four out of two hundred and ninety-eight patients 
with systematized delusions, and in fourteen out of six hundred and 
seventy cases of other forms of insanity. In 1885, Siemerling examined 
seven hundred insane women. He found an absence of the light reflex 
in three cases of senile dementia, in one of syphilitic dementia, in three 
tabetics with psychoses, in two epileptics, and in one case of systematized 
insanity; in all the other cases the light reflex was unaffected. In 
1896 he published a statistical study concerning nine thousand one hun- 
dred and sixty cases. He noted the absence of the light reflex in twenty- 
nine cases of tabes with psychoses, in seventeen cases of cerebral syph- 
ilis, in fifteen cases of alcoholic dementia, in nineteen cases of senile 
dementia, seven cases of paranoia, in four cases of hysteria, and in four 
cases of epilepsy. The above facts certainly justify the inference that 
disturbance of the light reflex is very rare among the insane. 

Moeli examined nineteen hundred patients. He found immobility 



FUNCTIONAL INSANITIES 509 

in fifty-six, but excluding organic cases, such as paresis, the percentage 
was only 0.8. Oebeker noted inequality in 2 per cent. Mignot says 
that, generally speaking, the percentage will be 2, and, since the differ- 
ence between paresis and other forms is so great, one can safely con- 
sider pupillary disturbances as belonging to paresis more than to any 
other form of mental disease. According to Verga and Castigliani, 
no changes of pupils are pathognomonic of insanity. 

Mignot analyzed one hundred and forty-four cases ; paresis was not 
included. He found inequality in 39 per cent., disturbed light reflex 
in 43 per cent., disturbed accommodation in 69 per cent., deformities in 
38 per cent., myosis in 17 per cent, mydriasis in 35 per cent. These 
results are analogous to the observations of Yerga and Castigliani. 

In psychoses following organic lesions of the brain, Mignot found 
marked pupillary changes in all. Thus in a case of maniacal excite- 
ment, following hemiplegia, the left pupil was larger than the right, 
the light reflex was absent and the pupils were deformed. In a case 
of melancholia with mental debility after hemiplegia in a diabetic, the 
right pupil was larger than the left and deformities were present. In 
a man suffering from paranoia, but also presenting a history of cerebro- 
spinal syphilis, the right pupil was larger than the left, there were 
loss of the reflexes to light and accommodation and deformities of both 
pupils. In a fourth case, a woman suffering from dementia following 
cerebral syphilis, the pupils were 5 millimetres in diameter, presenting 
incomplete light reflex and no convergence reaction. 

In senile dementia, Mignot found pupillary changes in all of the five 
cases examined by him. Thus one, a man of eighty, presented a myosis 
of 1 millimetre with absence of the reflexes to both light and accommo- 
dation. A second, a man of eighty-seven, presented pupils of 2.5 milli- 
metres with very sluggish reaction to light, no accommodation reaction, 
and deformities. In a third, a man of seventy-eight, presented pupils of 
2 millimetres with loss of both light and accommodation reactions. A 
fourth, a woman of eighty-one, presented pupils of 3 millimetres with 
likewise loss of light and accommodation reactions. A fifth case, a 
man of seventy-three, presented pupils of 5 millimetres with impaired 
light and accommodation reactions. Mignot also studied the pupillary 
changes in the precocious dementias in sixty-three cases. Of these 
sixty-three cases, nineteen had dilatation of the pupils ; nineteen pre- 
sented inequality. The light reflex was examined in fifty-three cases ; 
four times — i.e., in 7.54 per cent. — it was found lost and in twenty- 
nine — i.e., 54.71 per cent. — it was found altered. In the twenty-nine 
were included twelve cases in which variations of the liaht reflex were 



510 THE EYE AND NERVOUS SYSTEM 

noticed, — i.e., at certain times it was absent, at other times diminished, 
at other times normal. This variability appears to Mignot to be charac- 
teristic of the pupillary disturbances in dementia prsecox. In thirty- 
nine cases examined for the convergence reflex five presented dis- 
turbances. Finally thirty-one out of fifty-nine cases showed deformity. 
In detail the results are as follows : Inequality, 32.20 per cent. ; dis- 
turbed light reflex 62.26 per cent., disturbed accommodation reflex 12.81 
per cent., dilatation 30.15 per cent., and deformities 52.64 per cent. 

Marandon de Montyel's investigations show that abnormality of 
pupils occurs far more frequently in the chronic insane than in the 
acute. In twenty acute cases it was normal in 50 per cent, and abnor- 
mal in 50 per cent. In fifty-seven chronic cases it was normal in 5.2 
per cent, and abnormal in 94.8 per cent. He characterizes as chronic 
cases those that have a duration of two years at least of confirmed in- 
sanity. Even after this long lapse of time it is not extremely rare for 
recovery to follow. Paresis is doubtful only in the initial phase of 
the disease; after a duration of two years the symptoms are so pro- 
nounced as to leave no doubt as to the diagnosis. Therefore this rule 
can be of little avail for the differentiation of paresis. One out of 
two cases of acute insanity has an abnormal pupil. Disturbances and 
deformities were noted in the acute and chronic cases in the following 
percentages. Thus, in the acute, disturbances were noted in 15 per 
cent. ; deformities in 20 per cent, and disturbances and deformities to- 
gether in 15 per cent. In the chronic cases, disturbances were noted in 
50.8 per cent., deformities in 8.7 per cent, and disturbances and de- 
formities together in 35 per cent. The disturbances are nearly three 
times more frequent in the acute than in the chronic cases. In detail the 
special symptoms were found in the following proportions : 



Acute. Chronic. 

Per Per 

cent. cent. 

Inequality 3(15) 26(45.6) 

Myosis 0(0) 10(17.5) 

Mydriasis 0(0) 12(21 ) 

Deformities 7 (35) 25 (43.8) 

Alterations of light reflex 2 (10) 32 (56.1) 

Alteration of accommodation reflex 1(5) 20 (35 ) 

Bilateral identical alteration 0(0) 15 (26.3) 

Argyll-Kobertson pupil 2 (10) 19 (33.3) 

Inverse sign 1(5) 6 (10.5) 

Paradoxical reaction 0(0) 1 ( 1.7) 

Hippus 1(5) 2(3.5) 

Regarding the involvement of one or both eyes, de Montyel found 
that the deformities were unilateral in four of his acute cases and nine 






FUNCTIONAL INSANITIES 511 

of the chronic. In three acute cases and in sixteen chronic cases the 
deformities were bilateral. As regards the inequality of the pupils, he 
found a difference of less than a half millimetre in two acute and eigh- 
teen chronic, from one-half to one millimetre in none of the acute and 
six chronic, one millimetre and more in one acute and two chronic. 

In regard to deformities, de Montyel found them present upon one 
side in four acute and eight chronic cases ; upon the two sides in two 
acute and four chronic cases ; two deformities in one eye in none of 
the acute and one of the chronic cases ; two deformities in each eye in 
none of the acute and none of the chronic cases ; one deformity in one 
eye and two in the other in one of the acute and two of the chronic 
cases. 

These facts prove that one may find in the acute insanities de- 
formities of the pupils as pronounced as in the chronic insanities. De 
Montyel concluded also that the maximum deformities were more fre- 
quent in the acute than in the chronic cases. The acute cases would 
furnish a deformed pupil in the proportion of one in seven and the 
chronic one in more than a dozen. De Montyel further analyzed fif- 
teen of his acute and fifty of his chronic cases to show the evolution 
of the pupillary changes with the following result: He found the 
pupils normal throughout in 40.1 per cent, of the acute cases and in 
only 4 per cent, of the chronic cases. He found them abnormal 
throughout in 33.3 per cent, of his acute cases and in 90 per cent, of 
his chronic cases. It was first normal and later abnormal in 26.6 per 
cent, of his acute cases and in only 6 per cent, of his chronic cases. It 
was abnormal and later normal in none of the acute cases and in none 
of the chronic cases. According to this result, 40 per cent, of the 
acute cases have normal pupils as long as they are in the acute stage, 
while only 4 per cent, have normal pupils in the chronic stage. There- 
fore 36 per cent, of normal pupils of acute cases will become altered 
should these cases become chronic. In none of the cases, acute or 
chronic, examined by the author did he see pupils, once changed, again 
become normal, while in over one-fourth of the. cases pupils normal in 
the beginning presented changes later. 

Marandon de Montyel also made a study of the pupillary dis- 
turbances in summer and winter and he analyzed seventy-four in sum- 
mer and sixty-nine in the winter, a large proportion of the latter being 
cases studied by him in the preceding summer. In the summer he 
found 25.7 per cent, normal and 74.3 per cent, abnormal. In the win- 
ter he found 15.9 per cent, normal and 84.1 per cent, abnormal. The 
abnormal changes showed the following: Changes were present in 43.2 



512 



THE EYE A!NT> NERVOUS SYSTEM 



per cent, in the summer and in 42 per cent, in the winter. Deformities 
were present in 12.1 per cent, in the summer and 13 per cent, in the 
winter. Changes and deformities together were noted in 16.2 per cent, 
in the summer and in 29 per cent, in the winter, — that is, there was a 
noticeable increase in keeping with the duration and the tendency to 
chronicity. 

De Montyel's conclusions are as follows : In the majority of cases 
the pupils are altered in form and function in the insane ; while the 
changes are mostly slight, there is nevertheless a large proportion in 
which they are marked. They are almost always persistent and even 
increase in intensity. These facts de Montyel considers of great im- 
portance for the differential diagnosis of paresis. 

Marandon de Montyel has studied the pupillary disturbances and de- 
formities in the various forms of insanity. He makes an analysis of 
seventy-seven cases. The pupils were normal in 20 per cent, of mania, 
in 10 £>er cent, of melancholia and in per cent, of dementia, in 
per cent, of circular insanity and in 100 per cent, of insanity with sys- 
tematized delusions. They were abnormal in 80 per cent, of mania, 
in 90 per cent, of melancholia, 100 per cent, of dementia, in 100 per 
cent, of circular insanity and in per cent, of insanity with system- 
atized delusions. The abnormalities noted were as follows : 



Inequality 

Myosis 

Mydriasis 

Deformities 

Changed light reflex 

Changed accommodation reflex 

Argyll-Robertson 

Reversed sign 

Paradoxical pupil 

Hippus 



Mania 


Melancholia 


Dementia 


Per 


Per 


Per 


cent. 


cent. 


cent. 


6 (40 ) 


15 (37.5) 

8 (20 ) 

10 (25 ) 


6 (54.4) 





2 (18.1) 
1(9 





7 (46.6) 


17 (42.5) 


6 (54 ) 


5 (33.3) 


17 (42.5) 


8 (72.7) 


6 (40 ) 


10 (25 ) 


4 (36.3) 


4(26 


8 (20 ) 

2(5) 


6 (54.4) 


3 (20 ) 


2 (18.1) 





1 ( 2.5) 





1 ( 6.5) 


2(5) 






Circular 



Per 
cent. 

(40) 

(20) 
(40) 
(80) 
(20) 
(60) 



The unilateral or bilateral character of the symptoms is indicated 
in the table on the next page. 

The general conclusion can be drawn from it that bilateral changes 
predominate. All symptoms which show some tendency to inequality 
and deformities are to be noted. 

As to the method of involvement of the special reflexes of light and 
accommodation, de Montyel achieved the following results : In mania 



FUNCTIONAL INSANITIES 



513 



the reflex was at no time exaggerated, in melancholia he found it exag- 
gerated twice, in dementia never, never in circular insanity. He found 
it diminished three times in mania, eleven times in melancholia, three 
times in dementia and three times in circular insanity. It was much 



Myosis 

Mydriasis 

Deformities .... 

Light reflex 

Accommodation 

Paradoxical 

Hippus 



Mania 



Melancholia 



6 

7 

10 

17 

10 

1 

2 



P 
Dementia 



Circular 



2 





1 





5 


2 


8 





4 


















diminished in one case of mania, in one case of melancholia, in four of 
dementia, and in none of circular insanity. It was lost in one case of 
mania, three of melancholia, one of dementia, and one of circular in- 
sanity. 

The accommodation reflex was not found exaggerated at any time 
in mania, melancholia, dementia, or circular insanity. It was dimin- 
ished in four cases of mania, four of melancholia, four cases of de- 
mentia and one of circular insanity. It was much diminished in one 
case of mania, two of melancholia, in none of dementia and in none of 
circular insanity. It was lost in one case of mania, and in four of 
melancholia, in none of dementia and in none of circular insanity. 

De Montyel's conclusions are as follows: In systematized forms 
of insanity the pupils are constantly normal. 

In dementia the pupils are constantly abnormal. Identical changes 
are present in both reflexes to light and accommodation, while deformi- 
ties are present in general and are multiple ; a number of disturbances 
are present in the same patient and at the same time. There is a marked 
diminution of the light reflex. Deformities alone are rare. Paradoxical 
reaction is not found and there is no hippus. 

In circular insanity there is a constant abnormality of the pupils. 
There is a maximum of association of deformities and disturbances in 
the same individual. There is a tendency to unilaterality of deformities 
which in themselves are slight. There is no myosis, no paradoxical 
reaction, no hippus. 
33 



514 



THE EYE AND NERVOUS SYSTEM 



In mania, abnormalities are likewise found to occur frequently. 
There is a maximum of a weak accommodation and a minimum of a 
weak light reflex. There is no myosis, no mydriasis and no para- 
doxical reaction. 

Marandon de Montyel also studied the influence of age upon the dis- 
turbances and deformities of the pupils in the insane. He made an 
analysis of seventy-seven cases. Between the ages of twenty and thirty, 
16.1 per cent, were normal, while 83.9 per cent, were abnormal. Be- 
tween thirty and forty, 21.2 per cent, were normal, while 78.8 per 
cent were abnormal. Between forty and fifty, 11.1 per cent, were nor- 
mal, while 98.9 per cent, were abnormal. Between the ages of fifty and 
sixty, 11.1 per cent, were normal, while 88.9 per cent, were abnormal. 





20-30 


30-40 


40-50 


50-60 


Disturbances 


Per 
cent. 

5 (41.6) 
1 ( 8.3) 
4 (33.2) 


Per 
cent. 

10 ( 3.4) 
3 (10.3) 
9 (30.9) 


Per 
cent. 

7 (38.8) 
5 (27.7) 
4 (22.2) 


Per 
cent. 

10 (55.5) 



Deformities 


Disturbances and deformities 


6 (33.3) 





Inequality 

Myosis 

Mydriasis 

Deformities 

Light reflex 

Accommodation . . . 
Argyll-Robertson . . 
Reversed reaction . . 
Paradoxical reaction 
Hippus 



20-30 



Per 
cent. 

6 (50 ) 
1 ( 8.3) 

4 (33.2) 

5 (41.6) 
4 (33.2) 

1 ( 8.3) 

2 (16.6) 




1 ( 8.3) 



30-40 




( 3.4) 



40-50 



Per 
cent. 

6 (33.3 
3 (16.6 
2 (11.1 
9 (49.9 
8 (44.4 



(33.3 

(22.2 
( 5.5 
( 5.5 
( 5.5 



50-60 



Per 

cent. 

(27.7) 
(27.7) 
(11.1) 
(33.3) 
(61.1) 
(44.4) 
(38.8) 
(16.6) 



The frequency of variations in the light and accommodation reflexes 
is shown in the following tables : 



LIGHT REFLEX. 



Exaggerated. . . . 

Diminished 

Very diminished 
Lost 



20-30 



Per 

cent. 

1 (25) 
3 (75) 





30-40 



Per 
cent. 

63.8 
18.1 

(18.1) 



40-50 



Per 
cent. 

(12.5) 
(37.5) 
25 ) 
(25 ) 



50-60 



Per 
cent. 



7 (63.8) 

2 (18.1 

2 (18.1) 



FUNCTIONAL INSANITIES 



515 



ACCOMMODATION REFLEX. 



Exaggerated. . . . 

Diminished 

Very diminished 
Lost 



20-30 


30-40 


40-50 


Per 
cent. 


Per 
cent. 


Per 
cent. 













1 (100) 


4 (67.6) 
1 (16.2) 
1 (16.2) 


3 (51.4) 

1 (16.2) 

2 (32.4) 



50-60 



Per 
cent. 



6 (75 

1 (12.5' 

1 (12.5) 



De Montyel comes to the following conclusions : Age has no in- 
fluence upon the frequency of myosis, upon the number of disturbances 
present at the same time, upon the intensity of changes in the light 
and accommodation reflexes, upon the number, frequency, and extent 
and unilaterality of deformities. Age decreases the frequency and in- 
tensity of the inequality, but increases the frequency of changes in the 
light and accommodation reflexes and the frequency of the Argyll- 
Robertson pupil. It is especially before thirty and after fifty that the 
pupils are altered in the insane. 

In the immense majority of cases he found the pupils abnormal; 
it may be in their movements, in their shape, or it may be in both of 
these qualities at the same time. He also found that though the altera- 
tions are usually slight, they are in quite a noticeable proportion pro- 
nounced. Further, they are almost always persistent and often in- 
crease with time. 

Because of their detailed character, the results of Marandon de 
Montyel have in these pages been given special prominence, but not be- 
cause the author indorses them or accepts their accuracy. Unfortunately 
they do not bear criticism. Especially is this true in regard to the 
Argyll-Robertson pupil. Here his results must be wholly rejected. As 
Cestan and Depuy-Dutemps point out, mere weakness of the pupil in its 
reaction to light or variability of this reaction from day to day are not 
the signs which appertain to the true Argyll-Robertson symptom. The 
Argyll-Robertson symptom either exists or does not exist and is not sub- 
ject to daily variations. If we apply this rigid interpretation to the 
work of Mignot, for instance, we find that Argyll-Robertson pupil 
was noted by him only in one case of precocious dementia of the para- 
noid form, and in two observations in chronic alcoholics. This gives in 
reality a very small proportion, besides which the possibility of syphilitic 
infection in the above cases was mentioned by Mignot. Mignot very 
properly concludes that disturbance of the light reflex, such as is char- 
acterized by a complete and persistent abolition, offers an extreme prob- 
ability of a psychosis with organic lesions. 



516 THE EYE AKD NERVOUS SYSTEM 

Very different, on the other hand, are the statistics of Marandon de 
Montyel. In a fourth of his cases, he claims an Argyll-Robertson sign, 
and in a fifth, simultaneous weakness of the two reflexes, light and 
accommodation. These statements are surprising, for they are in com- 
plete opposition to current views. Marandon de Montyel gives the 
following proportions as to the existence of the Argyll-Robertson sign 
in the insanities: Mania, 26 per 100; lypemania, 8 per 100; de- 
mentia, 54 per 100; folie circulaire, 60 per 100; affirming thus the 
extreme frequency of the loss of the light reflex with preservation of 
accommodation to distance, exclusive of all general paralysis. 

Astonished at these results, Cestan and Depuy-Dutemps in turn ex- 
amined a large number of cases of insanity. The examination of the 
pupil was always practised in a dark chamber, with the light falling 
directly upon the macular region; illumination of the peripheral por- 
tions of the retina may provoke only a slight pupillary reaction even 
in normal persons. Among other things, Cestan and Depuy-Dutemps 
point out the necessity for rejecting procedures of examination which 
can accidentally put into play accommodation. If such precaution be 
not taken, the hand or the screen being suddenly withdrawn, and the 
eye fixing itself upon a distant point of illumination, may contract only 
very slightly or may indeed actually dilate. Cestan and Depuy-Du- 
temps noted only the exact Argyll-Robertson sign, — that is to say, the 
complete loss of the light reflex, with integrity of the accommodation 
to distance. They make mention of the fact that the investigation of 
this sign among the insane is sometimes impossible because of their agi- 
tation, their refusal to obey, or their dementia which interferes with the 
careful study of the light and accommodation reflexes. 

Cestan and Depuy-Dutemps excluded all cases of paresis. They 
studied fifty epileptics presenting various mental manifestations, thirty 
old men attacked with cerebral senile lesions, twelve cases of dementia 
prascox, three cases of intermittent mania, two cases of folie a double 
form, five cases of melancholia, sixteen cases of paronoia, sixteen cases 
of secondary dementia, two cases of neurasthenia with obsessions, one 
case of confusional insanity, one case of chronic chorea with mental 
symptoms, nine feeble-minded, nine imbeciles, and six idiots. All of 
the cases above mentioned were examined in a dark chamber by the pro- 
jection of a flame of light on the macular region. In every case was 
the reflex to light preserved. Thus the difference in the results of 
Cestan and Depuy-Dutemps and those of Marandon de Montyel is 
absolute ; it could not be greater. On the other hand, their results 
approximate those of Siemerling as well as those of Mignot. It is 



FUNCTIONAL INSANITIES 517 

probable that if Marandon de Montyel had tested his cases according 
to the method of Cestan and Depuy-Dutemps, and in addition had care- 
fully excluded all cases capable of being interpreted as tabes or paresis, 
or cases presenting gross lesions of the optic nerve, the difference in 
results would not have been so glaring. 

The results of Cestan and Depuy-Dutemps show, first, that in men- 
tal affections, as in diseases of the nervous system, the presence of the 
Argyll-Robertson pupil means organic disease, and secondly the finding 
should suggest a previous history of syphilis. How important this con- 
clusion is, we can only realize when we remember that among the insane 
an antecedent personal history is often impossible to obtain. 

Cestan and Depuy-Dutemps maintain that the Argyll-Robertson 
pupil consists in the complete absence of all contraction to light with 
persistence of the reaction to accommodation, no matter what may be 
the intensity of the light and in spite of the preservation of retinal sensi- 
bility. The sign is not susceptible of quantitative variations or grada- 
tions in intensity; it either exists or does not exist. Weakness of the 
light reflex should, of course, be noted, and if such weakness be asso- 
ciated with other organic signs of the symptom group of tabes or of 
paresis, it assumes some value. If such a weakness is the only symptom 
present, it cannot be interpreted as an Argyll-Robertson sign without 
loss of all precision. The degree of the pupillary reflexes is, of course, 
variable under physiological conditions, not to speak of functional dis- 
turbances, and great care should therefore be exercised in assigning a 
special value to a mere diminution of these reflexes. In the aged, for 
example, the pupillary reactions are normally feeble. They can scarcely 
be perceived in the case of intense mydriasis or myosis observed conse- 
quent upon drug intoxication or upon exhaustion or destruction „of the 
cervical sympathetic. Myosis of the pupils, for instance, is frequent 
among the aged, and by very reason of the narrowness and limited ex- 
cursion of the pupils a feeble reaction may be present. Further, a 
feeble reaction may be the effect of an alteration in the structure of the 
irides, without any lesion of the nervous system. Finally it should be 
remembered that a diminution of the light perception by change in the 
retina or by an optic atrophy is accompanied by a lessening of the light 
reflex. 

Not only is it difficult to reconcile the pupillary findings of Maran- 
don de Montyel with common clinical experience, but especially is this 
the case in his grouping of the insanities according to his findings. He 
groups the insanities as follows : First, a group formed by dementia and 
circular insanity in which abnormality of the pupils is constant in some 



518 THE EYE AND NERVOUS SYSTEM 

form or other; second, a group formed by mania and melancholia, in 
which, without being constant, abnormality of the pupils is the most 
common ; and third, systematized insanity in which abnormalities of the 
pupils are completely absent. Very curiously, he finds a constant ab- 
normality of the pupils in circular insanity, while it is merely common 
in mania and melancholia. If there is one fact which has been estab- 
lished within recent years and which is universally accepted, it is that 
mania, melancholia and circular insanity form one symptom group, and 
it seems unreasonable to suppose that circular insanity, the phases of 
which are indistinguishable from those of mania or melancholia, should 
present an especial peculiarity in regard to the pupillary reactions. It 
is only in regard to his findings in systematized insanity that Marandon 
de Montyel is in agreement with other observers. The suspicion is 
more than justified that his reason for finding no abnormalities in the 
pupillary reflexes in cases of paranoia is that such cases can be exam- 
ined with almost the same readiness as can normal individuals, a fact 
which is certainly not true of other forms of mental disease. 

When we turn our attention to studies by other authors, we find that 
evidence as to the absence of characteristic pupillary disturbances in 
the psychoses is practically universal. Thus the studies of Thomsen, 
already referred to, and made 'twenty years ago can be accepted to-day 
as still indicative of the actual facts as to the fixed pupil. Thomsen in 
1885 studied the pupillary phenomena in seventeen hundred insane. 
His conclusions are that by far the greater number of patients who 
show fixed pupil are paretic ; secondly, that the symptom is of great 
value, because it occasionally is present at the stage of the disease when 
the other symptoms of paresis are still but slightly developed; third, 
the fixed pupil in cases other than paresis is met with (a) in patients 
of advanced age (sixty to seventy years) and who present the symptoms 
of senile dementia; (b) in chronic alcoholics in rare cases; (c) in 
other forms of mental disease which present a history of previous 
syphilis or head injury; (d) under circumstances in which it is also 
observed in individuals psychically normal, — i.e., in tabes, multiple 
sclerosis, cerebral focal disease, brain syphilis, cerebrospinal meningitis, 
tuberculous meningitis, and oculomotor palsy; (e) in very rare cases 
without ascertainable cause. 

Thomsen concludes that the value in differential diagnosis of pu- 
pillary inequality and pupillary contracture without fixation of the 
pupils is very slight. Loss of the light reflex was present in 47 per 
cent, of his cases of paresis. In the non-paretic cases only 2.2 per cent, 
of such pupils were found. 



FUNCTIONAL INSANITIES 519 

That no special ocular symptoms are in accord with individual 
forms of insanity is proven not only by actual experience but also by a 
brief review of writings of various authors. Beginning with the manic- 
depressive group of insanities, we find an absence of all mention of char- 
acteristic pupillary reactions in the works of standard authors upon 
insanity. Mere variations in size, dilatation or contraction of the 
pupil are mentioned. Among writers who have especially studied the 
subject, we find similar statements. Thus Cowen states that in acute 
mania with great excitement, one may notice, though infrequently, hip- 
pus. Occasionally also a wide pupil is seen, but at the same time a 
normal reaction. Later, when exhaustion is pronounced, small pupils 
with a sluggish reaction to light may at times be seen. Again, E. M. 
Thomson has described a case of acute mania with dilated pupils and 
sluggish reaction to light. However, in the great majority of cases of 
acute mania, the pupils are quite normal in size, shape and reactions. 

In acute melancholia, there is usually nothing abnormal, but in 
cases with marked toxemic symptoms the pupil tends to become small 
and reacts sluggishly to light. 

Austin has seen inequality of the pupils in recurrent insanities and 
says that it passes from the right to the left when exaltation succeeds 
depression ( ?). Ritti has seen inequality as has also Regis. Of eleven 
cases examined by Mignot, only eight could be examined completely. 
Five times inequality was noted. One case showed a disappearance of 
the inequality during the quiet period. The light reflex was examined 
in seven cases. It was never lost and in only one case disturbed. It 
was normal in six. Six times convergence was obtained and was nor- 
mal. In three cases deformities were noted. Inequality was present in 
33 per cent., altered light reflex in 28 per cent., deformities in 5 per 
cent, and altered convergence reflex in none. 

Among cases of senile melancholia, Minck found spasmodic myosis 
in melancholia with hallucinations, while mydriasis existed in the ter- 
minal stage. Preston likewise found mydriasis. In melancholia with 
stupor, Ball and Regis found dilated pupils with loss of light reflex. 
Austin says that in old people with melancholia the right pupil is often 
larger and more sluggish than the left. Mairet and Athanassio write 
that loss of the accommodation reflex and persistence of the light reflex 
deserve to take a place in the symptomatology of melancholia; it is 
quite evident, however, that abnormalities of accommodation must be 
referred to the inattention and psychic indifference of the patient. In 
twelve patients examined by Mignot, the pupillary disturbances pre- 
sented special features : four showed inequality, in two the inequality 



520 THE EYE AND NEKVOUS SYSTEM 

was not constant but was always due to changes on the same side. With 
the exception of one patient, in whom the light reflex could not be 
investigated and in another in whom the reflex was normal, the pupils 
reacted poorly. The reflex to convergence was normal in seven cases. 
In five others the results varied at different examinations. Deformities 
existed in eight cases. No relation was found between the state of the 
pupils and the variations in the mental condition. 

In paranoia, ocular phenomena are quite constantly absent. This 
is true not only of the pupillary reactions, but also of the fundus. 
Thus, J. C. Connell, who studied the eyes in paranoia, states that no con- 
stant changes were found. Conditions of depression and exaltation do 
not furnish corresponding appearances in the fundus. Connell agrees 
with the great mass of observers when he states that when affections of 
the eye are present, they are due to some material cause such as tabes, 
syphilis or albuminuria in the course of which the insanity develops. 
Of five cases studied by Mignot, only one had pupillary changes but this 
case also showed cerebral arterio-sclerosis. Of seventeen degenerates 
affected with systematized delusions of persecution with hallucination, 
there were found one case of inequality, one of myosis with sluggish 
reaction to light, five with a diminished light reflex, and only five with 
deformities. Inequality was present in 54 per cent., diminished light 
reflex in 27 per cent., myosis in 54 per cent., deformities in 36 per cent., 
disturbances of convergence in none. 

In delirium, confusion and stupor characteristic changes are likewise 
absent. Beyond variations in the size of the pupils, nothing is noted. 
This is true of even delirium grave as witness the case reported by 
Pritchard. Occasionally, however, the pupils may be unequal as in the 
case of amentia reported by Pilcz, in which the right pupil was larger 
than the left. At times sluggishness of reaction is noted as in the case 
reported by A. N. V. Johnson, who describes a case of acute confusional 
insanity following influenza in a woman of 43. The pupils were mod- 
erately dilated and sluggish to light and accommodation. 

Alcoholic insanities are exceedingly interesting but likewise show 
nothing characteristic. Thus, Minck found in delirium tremens, myosis 
and during the terminal phase, mydriasis. Ball and Kegis noticed not 
only frequent myosis, but also inequality. In the course of the attacks 
of delirium with hallucinations which lead the chronic alcoholic toward 
dementia, Magnan has often seen inequality of pupils. The latter he 
has also seen in degenerates who had attacks of delirium under the 
influence of alcohol. Krafft-Ebing mentions also dilatation of the pupils. 
Mignot found that of seven patients affected with delirium and hallu- 



FUXCTIOXAL IXSAXITIES 521 

cinations, examined at the beginning or during the disappearance of 
the symptoms, only two showed no pupillary disturbances. In four 
cases there was found inequality; in one of them the inequality disap- 
peared under the influence of abstention from alcohol. In two patients 
the light reflex was diminished; in one it subsequently again became 
normal. In four patients deformities were observed. Fourteen per 
cent, of Mignot's acute cases presented inequality; fifty-seven per cent, 
diminution of the light reflex, fourteen per cent, deformities, while none 
of them presented alterations of convergence. Among the chronic alco- 
holics studied by him, one only had normal pupils ; six showed inequal- 
ity; two suffered from loss of the light reflex; five from diminished 
light reflex. In one case a diminished convergence reflex was observed, 
in another myosis with inequality. All except one showed irregular 
pupils. 

Raimann in 1901 published a study of alcoholic palsies of ocular 
muscles. He states that ocular muscles are not infrequently paralyzed 
in alcoholic cases characterized by Korsakow's psychosis. Statistically 
he found that 30 per cent, of such cases presented ocular troubles, such 
as inequality and impaired reaction of the pupils. A. Westpkal noted 
paralysis of the eye muscles in a case of Korsakow's psychosis. The 
case was that of a man of thirty-six. The eyes could not be moved 
upward or downward. Visual acuity was diminished, the left pupil 
was larger than the right. The light reflex was sluggish on both sides, 
while the consensual reflex was also slight. The ophthalmoscopic 
examination revealed a slight pallor of the disks on the temporal sides. 
J. Turner has made an analysis of twelve cases of Korsakow's psycho- 
sis. In eight cases there were no eye symptoms. In four the following 
conditions were noted. In the first, a woman of 53, the pupils were 
equal and contracted, and either rigid to light and accommodation 
or at most reacted very slightly. In the second case, a man of 29, 
the pupils were equal and reacted to light with a restricted range. In 
the third case, a woman of 39, the right pupil was larger than normal. 
In a fourth case, a woman of 10, the right pupil was larger than the left ; 
both pupils reacted to accommodation, but the left seemed rigid to light, 
and the right reacted very slightly to both direct and consensual light 
stimulation. 

Regarding the pupillary reflexes of dementia pra?cox, definite 
knowledge is still wanting. According to Kraepelin, the pupils are fre- 
quently strikingly dilated, especially in the stages of excitement. This 
I think is undoubtedly in accord with common experience. Bumke, who 
has records of thirty-three cases, of which nine were hebephrenics, 



522 THE EYE AKD NERVOUS SYSTEM 

twenty-two catatonics and two of the paranoid form, states that in all 
of them the pupils were throughout larger than normal. They averaged 
six and one-half mm. Rarely was the pupillary diameter less than five 
and one-half mm. In two cases, however, nine mm. were measured. 
Inequality of the pupils was observed only three times and a marked 
difference between the right and left, over two mm., was only observed 
once. He observed remarkably rapid changes of the diameter of the 
pupils in six of his cases. Such indeed, for example, that upon one occa- 
sion five and one-half mm. were measured and several days later under 
the same conditions seven mm. were measured. Bumke does not venture 
to assign a reason for this phenomenon. A difference as to the size of 
the pupils does not seem to obtain among the three forms of dementia 
prsecox. 

Changes in the light reflex are doubtful in character. Kraepelin, 
for instance is unwilling in view of the possibilities of error in the exam- 
inations, to make definite statements as to this point. Bumke believes 
as far as catatonia is concerned, that if disturbances of the light reflexes 
are present, they are always insignificant. He has frequently received 
the impression that the pupillary contraction in catatonic cases comes on 
more suddenly and disappears more suddenly than in normal cases. 
He does not, however, regard the symptom as of value. He regards the 
reflex as active, but does not venture to say that it is regularly exag- 
gerated. 

The accommodation reaction appears to be entirely undisturbed. 
This is also true as to the relationship between the light and conver- 
gence reaction. On the other hand the Piltz reaction of the pupil fre- 
quently departs from the normal. It seems to be more pronounced and 
more readily observed than normally. For instance, in eighteen of 
Bumke's cases and in all of the catatonics, a contraction from two to 
three millimetres was readily observed. Bumke does not venture to give 
an explanation of this condition. 

The condition which Bumke would regard as typical for dementia 
prsecox is the absence of the psychic reflex, the reduction of the pupil- 
lary motility and the absence of the reflex dilatation to sensory stimu- 
lation. In hebephrenics, the psychic reflexes — i.e., the movements of 
the iris in response to the play of concepts and emotions — were always 
absent in cases in which the disease had lasted for a relatively long time 
and when distinct psychic enfeeblement had become established. In 
two cases, Bumke noted the presence of these reactions at an early stage 
and later noted their disappearance. He states that he has never ob- 
served a return of this reflex in this form of dementia prsecox. . 



FUNCTIONAL INSANITIES 523 

In melancholia, abnormalities are very frequent but not constant. 
There is frequently a tendency to unilateral myosis or unilateral 
mydriasis, furthermore none of the patients that he observed escaped 
without persistent psychic defect. In three of his cases, the reflex 
dilatation to sensory stimulation Avas preserved at least when very pain- 
ful stimuli were employed ; twice such preservation was temporary and 
•once it was permanent. In one of these patients, this reflex dilatation 
was preserved in the beginning of the disease only. In another patient, 
in whom it had been absent, it appeared when the psychic symptoms 
improved. 

In the catatonic form of dementia prsecox, Bumke has never been 
able to confirm the existence of the psychic reflexes. In six of his 
twenty-two cases, however, he was able to bring about a feeble dilatation 
of the pupils through very painful sensory stimulation. In one of the 
two cases of the paranoid form, both the psychic reflexes and the reflex 
dilatation to irritation was wanting. In the other the pupillary psychic 
reflexes were wanting while the sensory stimulation was still active in 
producing dilatation of the pupil. 

Bumke also tested the action of cocaine, hematropine and eserine in 
dementia prsecox. Definite results, however, were not obtained. Hom- 
atropine and eserine act exactly as with healthy individuals. Cocaine 
appeared at times to have a slight action. However, nothing definite 
can be said concerning this point. 

It would appear, then, that in dementia praecox definite changes in 
the pupillary reactions are not found save, first, such as can be attributed 
to defective innervation, — i.e., the dilatation of the pupil; and sec- 
ond, such changes or deficiencies of action as depend on diminished 
psychic activity. Definite changes in the light and accommodation re- 
flexes are not present. Loss of the psychic reflexes occurs of course in 
other forms of dementia, notably in paresfs. Here, however, definite 
•changes in pupillary reactions make their appearance either early or 
concomitantly. 

In secondary dementia, the pupils are usually quite normal, though 
in middle-aged cases there is not infrequently a sluggish reaction to 
light with a rather small pupil. In senile dementia, the only symptom 
noted is, as a rule, a small pupil with a distinctly sluggish reaction to 
light. Occasionally unequal pupils are found. In dementia depending 
upon gross lesions, such as tumor, softening or meningitis, the pupillary 
reactions may or may not be altered. If changes be present, they 
depend, other things equal, upon the site and the nature of the lesion 
and these are so various that they cannot be considered here. 



524 THE EYE AND NERVOUS SYSTEM 



SUMMARY. 

In summarizing the ocular symptoms of the functional psychoses, 
we have to repeat the opening paragraph of this section. First, the ocu- 
lar symptoms are relatively unimportant and possess but little clinical 
value ; second, there are no pupillary changes which are peculiar to any 
mental disease. 

In all cases in which actual and persistent pupillary changes are 
found to exist, we can predicate the presence of grave organic 
disease. 

Many of the elaborate studies that have been made have failed of 
definite result. With the exception of the loss of the psychic reflexes 
noted in dementia prsecox and in other forms of dementia, all of the 
elaborate studies as to pupillary changes in the functional psychoses 
may be regarded as having established nothing save the single fact that 
in stages of excitement and stages with marked diminution of inner- 
vation, the pupils become dilated. However, even this generalization 
can be regarded as having only a limited application. It is true that 
a dilated pupil is particularly frequent in the affective psychoses, but 
even here no rule can be formulated. 

Because of the etiological relationship between degeneracy and in- 
sanity, we meet with — though it must be admitted rarely — morpholog- 
ical peculiarities. Though rarely present, they are always significant 
of arrest or malformation. We may, for instance, meet with unusual 
prominence of the supraorbital ridges, unusual narrowness of the space 
between the two eyes, or on the other hand unusual width of this space, 
so that the eyes seem to be too close together or too widely separated. 
Again, the palpebral fissures are sometimes found unusually wide or 
unusually narrow, or a little too straight or perhaps somewhat oblique or 
Mongolian in direction. Rarely gross defects, such as coloboma, may be 
noted. The iris may also be congenitally absent, or there may be several 
openings, or the pupils may be abnormally situated. Again, there may- 
be irregularities of the pupils or perhaps an imperforate iris, or there 
may be anomalies of coloring, such as patches of pigment, or albinoism 
may be noted. The pupils may be congenitally asymmetrical and un- 
equal. Congenital disease of the crystalline lens may be noted. While 
the anomalies and malformations here mentioned should be borne 
in mind, it is on the whole surprising that they are not met with 
more frequently, especially when we bear in mind the obvious re- 
lationship existing between some forms of mental disease and defective 
organization. 



CHBONIC ALCOHOLIC INTOXICATION" 



525 



CHRONIC ALCOHOLIC INTOXICATION. 

The ocular symptoms of chronic alcoholic intoxication consist chiefly 
in disturbances of vision. It has long been known that the chronic 
abuse of alcoholic stimulants may result in a more or less pronounced 
amblyopia. It would appear that this amblyopia is dependent upon an 
interstitial inflammation followed by an atrophy of the nerve-fibres. 
The early symptoms are those of dimness of vision, and it is worthy of 
note that the patient can read better in a somewhat subdued light, — 
that is, a light that is not very bright. Vision is, as a rule, somewhat bet- 
ter at night. There is present a central scotoma for colors and for 
white. The impairment of sight comes on gradually. Visual acuity is 




25&^ TTT^ 285 



Fig. 1. — Absolute central scotomata ; peripheral boundaries of the field normal in extent. 

(De Schweinitz. ) 



below normal in both eyes. Occasionally, however, the amblyopia 
comes on rapidly, or there may be a sudden accentuation of symptoms 
attendant upon renewed excesses or upon undue fatigue. According 
to Uhthoff, the scotomata are at first for red and green. Terrien states 
that the first color affected is green, and it is only much later that blue 
and yellow disappear. The ophthalmoscopic examination reveals in the 
beginning, even when vision is already much disturbed, a normal eve- 
ground, though in some cases there may be a hypersemia of the papilla. 
When, however, the scotoma for colors and for white is extensive, atrophy 
of a part of the papilla may be noted. According to Uhthoff, the changes 
which belong to chronic alcoholic intoxication as well as those of tobacco 
and arsenic, consist of a partial retrobulbar neuritis. The process re- 
mains limited to the papillomacular bundle of fibres. The inflamma- 
tory manifestations in the papilla are usually slight, while the secondary 



526 THE EYE AND XEKVOUS SYSTEM 

atrophy is usually confined to the temporal half. There is a pallor of 
the temporal portion of the papilla, or pallor of the lower and outer 
quadrant. According to Uhthoff, there is a pallor of the temporal side 
of the papilla in 63 per cent, of the cases. A complete optic atrophy 
is very rare. 

According to all investigators — Erisman, Leber, Samuelsohn, JSTet- 
tleship, Vossius, Magnan and UhthofT — there is an inflammation of the 
interstitial connective tissue of the optic nerve analogous to that which 
takes place in the liver in alcoholic subjects. The fibres of the macula 
seem especially to suffer from the alcohol. The change consists in an 
overgrowth of the interstitial connective tissue, which is followed by a 
secondary degeneration and atrophy of nerve-fibres. Xoel, however, 
thinks that the primary change is after all to be sought for in the nerve- 
fibres and that this is secondarily followed by an overgrowth of inter- 
stitial connective tissue, — that is, he believes that there is a parenchy- 
matous neuritis, with later a secondary connective tissue prolifera- 
tion and atrophy. 

Uhthoff found 0.43 per cent, of alcoholic amblyopia in thirty thou- 
sand eye cases. Adler found 0.42 per cent, in seventy-one thousand 
eight hundred and sixty-five cases. It is remarkable that, in three 
hundred and four cases of alcoholic amblyopia, only four occurred in 
women. 

The percentage of cases at various ages Adler found as follows: 
Between thirty and forty, 18 per cent. ; between forty and fifty, 46 per 
cent. ; between fifty and sixty, 26 per cent., and between sixty and 
seventy, 8 per cent. 

Fleet reports a case occurring in a woman who, after imbibing a 
large quantity of alcohol, became blind. Eight weeks after the onset of 
the blindness, an examination revealed the following: The pupils were 
dilated ad maximum; there was no reaction to light; vision in the 
right eye consisted in the ability to count fingers at a distance of two 
feet, while in the left eye there was barely a perception of light ; the 
optic nerves were dead white, while the blood-vessels were small. Fleet 
says that chronic subacute retrobulbar neuritis in persons addicted to 
alcoholic beverages is not uncommon, and we may expect a gradual or 
passive inflammation of the optic nerve from a gradual alcoholic poi- 
soning. It results from a bilateral interstitial inflammation of the 
orbital portions of the optic nerves. The first symptoms are those of 
failing vision ; the patient can see better in a subdued light ; there is 
a peculiar stare ; the pupils are slightly dilated ; the light reaction 
is sluggish; the peripheral vision is better than the central, and there 



CHRONIC ALCOHOLIC INTOXICATION 527 

is inability to distinguish red and green in the centre of the visual field. 
In acute retrobulbar neuritis, we find the pupils widely dilated; there 
is no light reflex, and there is no perception of light. 

In acute alcoholism, visual phenomena are infrequent and with rare 
exceptions they are transient in duration. Interference with the 
visual field is almost never noted. On the other hand, paresis of ex- 
ternal ocular muscles and disturbances of the pupillary reactions may 
be met with. Now and then, owing to damage to the nuclei of the 
nerves supplying the extrinsic muscles, a permanent paralysis may 
result. Blindness has, very rarely, been noted in acute alcoholism, but 
usually with a recovery of vision. Noel states that in acute alcoholic 
amblyopia there may be a rapid loss of sight in both eyes following the 
excess or overindulgence. Blindness may be complete from the start, 
but sight may later be recovered. The pupils may be much dilated and 
motionless. 

The reflex to light may be impaired or lost during profound alco- 
holic intoxication but may reappear subsequently. Hans Gudden 
thinks that the reaction of the pupil to light is a good test for determin- 
ing the state of consciousness. The light reflex may, of course, be 
impaired or even lost in chronic retrobulbar neuritis. 

Raimann has made a study of the ocular palsies in alcoholism, and 
believes that the ophthalmoplegia occasionally noted is due either to a 
neuritis or to a polioencephalitis superior. In two cases reported by 
him, there was in the first a right ophthalmoplegia, both internal and 
external ; on the left side the abducens only was paralyzed ; the palsies 
gradually disappeared. In the second case, the pupils were small and 
of the Argyll-Robertson type. There were conjunctivitis, nystagmic 
movements, ophthalmoplegia interna, and double abducens paralysis. 
The autopsy revealed a hemorrhage in the aqueduct of Sylvius. Alto- 
gether, about twenty-three cases of polioencephalitis superior have been 
reported in which alcoholism was present. Out of forty-four cases of 
alcoholism studied by Raimann, there were only seven with distinct eye- 
muscle palsies. Although there were polyneuritis symptoms in these 
cases, Raimann is of the opinion that neuritis cannot be considered as 
explaining the palsies of the eye muscles, but believes the latter to be 
central in their origin. 

A case with autopsy, reported by Bonhoeffer, corroborates this view. 
Of the extrinsic muscles the external rectus is the most frequently 
affected. In slight cases there is usually merely a weakness of this 
muscle. Nystagmic movements may be noted when the patient at- 
tempts to look toward the side of the paralyzed muscle. Next in fre- 



528 THE EYE AND NERVOUS SYSTEM 

quency is involvement of the internal rectus. Ptosis also is not infre- 
quently noted. These palsies usually come on suddenly. 

Bonhoeffer has reported a case of alcoholism with focal cortical symp- 
toms in which, hefore death, disturbances in the eye muscles occurred. 
Jolly has reported twenty-one cases of alcoholism, of which two pre- 
sented paresis of eye muscles. 

Thomsen examined six hundred and seventy alcoholics. He found 
no light reaction on either side in nine cases, and in one case loss of 
the light reaction upon one side. There was sluggish reaction on both 
sides in fifty-seven cases and on one side in eleven cases. In 12 per 
cent, of his patients there were pupillary disturbances. Siemerling 
found, in twenty-nine cases of alcoholism in women, only one case of 
loss of light reflex. Moeli, in a series of cases, found only four of per- 
manent and three of temporary loss of the light reaction. 

Raimann's experience, based upon two hundred and seven cases of 
alcoholism, yields twenty-five per cent, of disturbances of the innerva- 
tion of the eye muscles. Among all of the eye symptoms, pupillary 
disturbances were the most frequent. Myosis, mydriasis, or slow and 
unequal reaction of the pupils, may be present. Pupillary involvement 
may, of course, be present at the same time that the extrinsic muscles 
are involved. Raimann believes that alcoholic ophthalmoplegias are 
almost all of central origin. The possibility of a polyneuritis of the 
nerves supplying the eye muscles must, however, be admitted. The 
prognosis of alcoholic ophthalmoplegias is uncertain. 

As might be expected, amblyopia not infrequently occurs in per- 
sons who are at one and the same time the victims of chronic alcoholic 
and chronic tobacco poisoning, and in such cases it may be difficult and 
indeed impossible to separate the two conditions. It is well known that 
both alcohol and tobacco may independently — and this is true also of 
arsenic — cause an amblyopia with central scotomata and temporal dis- 
colorations of the papilla, while the peripheral vision is intact. 

OCULAR SYMPTOMS OF LEAD INTOXICATION. 

Blindness, as a result of lead intoxication, has long been known, 
though it was not until Grisole, in 1835, and shortly after Tanquerel 
des Planches, in 1839, described the cases studied by them that the role 
of lead in the production of blindness, together with lead colic and other 
general symptoms, was properly appreciated. 

As in the case of alcohol, chronic lead poisoning may give rise to 
both amblyopia and disturbances of muscle innervation. The eye symp- 
toms are, however, always (or nearly always) preceded by the general 



LEAD INTOXICATION 



529 



symptoms of lead-poisoning. The amblyopia presents typical central 
scotomata. At the same time, there may be concentric contraction of 
the fields for form and colors. Not infrequently the scotoma assumes 
the form of a hemianopsia. 

Actual organic changes — such as optic neuritis, neuroretinitis, optic 
atrophy, or changes in the vessels (perivasculitis) — may supervene. 
Exceptionally the amblyopia is recovered from and is then probably due 
to an anaesthetic effect of the poison, rather than to organic changes. 
Ophthalmoscopic appearances, as may be surmised, vary from nega- 
tive findings or mere vascularity of the nerve head to the decided 
organic changes just detailed. Of sixty-four cases analyzed by de 
Schweinitz, thirteen presented optic neuritis, four neuroretinitis and 
seventeen optic-nerve atrophy. In seventeen cases of the sixty-four, 



L.E. 




defect, 



Fig. 2.— Field of vision from Uhthoff's case of lead-amblyopia with ophthalmoscopic appearances 
resembling intoxication-amblyopia (temporal half of papilla discolored). Normal form field and abso- 
lute central scotoma. (De Schweinitz.) 



blindness was noted without description of the ophthalmoscopic appear- 
ances. De Schweinitz thinks that no doubt in many of these atrophy 
or neuritis was present, and he regards Stood's result of fifty per cent, 
of organic nerve change, based on a study of thirty-four cases, as not 
too high. Some authors — for example, Hirschberg and Knies — con- 
sider lead amaurosis identical with uraemic amaurosis. Most writers 
regard the influence of lead upon the optic nerves as direct. Others, 
like Oliver, regard the action of lead upon the retina as anaesthetic ; 
while others still, such as Jaccoud and Weber, lay stress upon the cir- 
culatory disturbances. Elschnig has reported a case in which the cir- 
culation of the retina seems to have been at fault ; there was an arterial 
spasm and the blindness was attributed to the ischa?mia. 

A clear conception of the visual and ophthalmoscopic findings is 
afforded by the following illustrative cases taken from the literature : 
34 



530 THE EYE AND NERVOUS SYSTEM 

Westphal, in 1888, reported a case in which there were hemianop- 
sia, cataract of one eye, and optic neuritis in the other. Hertel, in 
1890, reported a case in which there was a left homonymous hemianop- 
sia, narrowness of the retinal arteries, and sluggish reaction of the 
pupils. Elschnig reported, in 1898, a case of temporal hemianopsia. 
Bihler reported, in 1899, the case of a compositor, thirty-nine years of 
age, in whom in the left eye the visual field of the nasal side was lost 
and in the right eye the temporal visual field. The hemianopsia was 
partial, but there was complete hemiachromatopsia. There was slight 
nystagmus and the pupils were narrow on both sides. Taylor, in 1898, 
reported the case of a woman, twenty years of age, in whom there was 
failing sight with marked central scotomata in both eyes. There was 
white optic atrophy on the left side, while the right papilla was ap- 
parently normal; there was here an amblyopia without obvious tissue 
change. In two cases of lead poisoning, reported by de Schweinitz, 
there was partial optic-nerve atrophy together with central scotomata. 
In the first case, that of a house painter, aged thirty-five, vision was re- 
duced in both eyes. The disks were nearly circular, with a sharp cen- 
tral excavation ; there was moderate chorioidal disturbance in the cir- 
cumference ; there were central scotomata with contraction of the visual 
field of the temporal side of the right eye. In the left eye the visual 
field was normal ; there were no muscle palsies. In the second case, 
also a painter, aged fifty-nine, besides marked impairment of vision, it 
was noted that both optic nerves were grayish, the vessels were small 
and there were present central scotomata for red and green. Later, 
there was a general grayish-white atrophy and a marked perivasculitis,, 
with marked concentric contraction of the visual fields, especially upon 
the right side, and decided contraction of the red field and blindness 
for green. 

Eye symptoms due to lead poisoning are surprisingly rare. Tan- 
querel des Planches found only twelve cases of amblyopia in one thou- 
sand two hundred and seventeen cases of lead poisoning. According to 
de Schweinitz, also, lead amblyopia, compared with the number of 
cases of chronic lead poisoning, is infrequent. Packard examined forty- 
eight workers in lead; thirty-three of them showed symptoms of the- 
toxic influence of lead, but no case was found of amblyopia, traceable 
to lead. Again, lead amblyopia is rare in comparison with the whole 
number of ocular diseases, although it is difficult to give exact figures. 
De Schweinitz states that among more than twelve thousand cases of 
ocular disorders recorded at the Jefferson Hospital and the Philadel- 
phia Polyclinic during five years, only three cases of optic-nerve or 
retinal changes attributable to lead were found and of these two 



LEAD INTOXICATION 531 

were doubtful. Further, the number of amblyopias, among the total 
number of toxic amblyopias, is small. UhthofT reports two hundred 
and four cases of retrobulbar neuritis among thirty thousand patients ; 
of these one hundred and thirty-eight were toxic in origin, but only 
one was due to lead. The ocular manifestations of chronic lead poison- 
ing may consist of transient amblyopia due to the anaesthetic effect 
of lead upon the optic nerve and retina, or of a permanent amblyopia 
due to retrobulbar neuritis. The latter may terminate in permanent 
atrophy, optic neuritis, or neuroretinitis specifically due to lead and 
which last may in turn be followed by optic-nerve atrophy. Finally, 
amblyopia may be due to a vasculitis of the retinal vessels. 

The least known of the ocular symptoms due to lead poisoning are 
the disturbances of the movements of the eyes and the reactions of the 
pupils. De Schweinitz states that chronic lead poisoning is a well rec- 
ognized cause of paralysis of the external ocular muscles. According 
to Schroeder, the abducens is the one most frequently involved, a con- 
dition which is, perhaps, analogous to the palsies of the extensors of 
the extremities. 

Elschnig has described seventy-six cases of palsies of the motor 
nerves of the eye. Renaut, Galezowski, Dejerine-Klumpke, Manna- 
berg, Lilienfeld, Lagrange, and lately Aurant and Burnot, have de- 
scribed double palsies of the external recti. According to Elschnig, 
there are two varieties of ocular palsies occurring in the course of lead 
intoxication : First, palsy of central origin, associated with vertigo, 
headache, and vomiting, the cases terminating in rapid death ; second, 
palsies of peripheral origin. The latter are more frequent, less rapid in 
their onset, and are less fatal. Ptosis, strabismus and diplopia may be 
present. The third nerve may be involved in its entirety, or the paraly- 
sis may affect only one of its branches. Isolated pupillary and accom- 
modation disturbances do not frequently come under "observation. Elsch- 
nig twice found unilateral palsy of the sympathetic, narrowness of the 
palpebral fissure, and sluggish light reaction. In one case he found 
symptoms of irritation of the sympathetic. In a case reported by Red- 
lich there was pupillary rigidity to both light and convergence, while 
vision was preserved. This finding, however, must be regarded as ex- 
ceedingly rare, — so rare, indeed, as to be open to doubt. Crooke has 
placed on record a case of lead poisoning accompanied by delirium, 
coma, and convulsions, followed by blindness. In this case the pupils 
were at first contracted and later dilated. According to de Schweinitz, 
the iris movements vary with the condition of the optic nerve and retina 
from absolute immobility to normal reaction. 



CHAPTER XIII. 

AMAUROTIC FAMILY IDIOCY AND CEREBRAL PALSY OF 

CHILDHOOD. 

By B. SACHS, M. D. 

AMAUROTIC FAMILY IDIOCY. 

The above title was given by me to a rare disease affecting several 
members of the same family, and characterized by a distinct lack of 
mental development, by a progressive weakness of all the muscles of the 
body, and by a defect in vision (associated with changes in the macula 
lutea and optic nerve atrophy) terminating in complete blindness. The 
disease is generally fatal, the children dying as a rule in a condition of 
complete marasmus before the end of the second year of life. 

History. — In 1881 Waren Tay described a case presenting " sym- 
metrical changes in the region of the yellow spot in each eye of an 
infant. The child was twelvemonths old. It was deficient in holding 
up its head or moving its limbs. There was weakness but no absolute 
paralysis of any part. Its cerebral, development was slow and poor. At 
the first examination, March 7, 1881, the optic disks were apparently 
healthy, but in the region of the yellow spot of each eye there was a con- 
spicuous, tolerably diffuse, large white spot more or less circular in 
outline, and showing at its centre a brownish-red, fairly circular spot 
contrasting strongly with the white patch surrounding it. This central 
spot did not look at all like a hemorrhage, nor as if due to a pigment, 
but seemed a gap in the white patch, through which one saw healthy 
structures." The author likened these appearances to those one is 
familiar with in cases of embolism of the central artery of the retina. 
He believed the changes in the retina to be " possibly congenital." 
Five months later another examination was made, showing that the 
disks had become atrophied, but that the changes in the macula lutea 
were the same as before. In the same family, according to Waren 
Tay's later reports, three similar cases had occurred, each one of the 
children presenting ocular symptoms and exhibiting physical condi- 
tions that were similar in all respects, and all three dying before the 
age of two years. This peculiar ophthalmoscopic finding was noted by 
Magnus, Goldzieher, Wadsworth of Boston, Hirschberg of Berlin, and 
H. Knapp. 
532 



AMAUROTIC FAMILY IDIOCY 533 

In 1887, without any knowledge of the cases described by the 
oculists, I published the history and the post-mortem record of a patient 
suffering from what appeared to be a peculiar form of idiocy associated 
with blindness. The family character of the affection was not evident 
until a sister of my first patient became similarly affected. In still 
another family I saw another instance of this affection, and received 
the history of three other children who had been afflicted with and had 
died of this disease. 

Kingdon, of Nottingham, called attention to the fact that the rare 
condition reported by the oculists was part of the disease which I had 
described. In 1894 Carter collected all cases of this disease known up 
to that time, and in 1896 I was able to give a list of nineteen cases of 
which eight had come to my own notice. Since the publication of this 
last paper other cases of this sort have been described and published 
by American and some European writers. In Europe cases have been 
published by Kingdon and Russell, 1 Higier, Ealkenheim, Shaffer, Frey, 
and others. In 1901 Falkenheim analyzed a series of sixty-four cases 
and since that time others have been reported both here and abroad. 

Symptomatology. — As a rule, the children affected with this disease 
are born at full term and apparently in perfect health. They do well 
until the first three to six months of life, when they become listless and 
apathetic, move their limbs very little, and show the first signs of visual 
disturbance which ultimately leads to blindness. The child is not able, 
as the months go on, to hold up its head or to sit up. Its muscles are 
either flaccid or spastic ; the reflexes are normal, a trifle subnormal, or 
exaggerated. In some cases there is an unusual sensitiveness to touch 
and to sound (hyperacusis), the child being startled by the slightest 
noise occurring in the room. Convulsions are present in some cases 
but are not an integral symptom of the disease. All the functions of the 
body are in a low state of activity. The children are subject to frequent 
bronchial attacks and soon show gastro-intestinal disturbances. An 
examination of the fundus reveals the peculiar condition so well de- 
scribed by Tay. (See Fig. 1). There is a gradual increase of all the 
symptoms, the mental defect becomes more and more noticeable, the 
palsy more extreme, complete blindness is established and the child 
gradually lapses into a condition of marasmus in which it dies as a rule 
before the end of the second year. The chief symptoms may be summed 
up as follows: (1) Mental impairment observed during the first months 
of life and leading to absolute idiocy; (2) paresis or paralysis of the 

1 Russell has used the designation "Infantile Cerebral Degeneration;" the term 
is unsatisfactory and wholly inadequate. 



534 THE EYE AND NEKVOUS SYSTEM 

greater part of the body, and this paralysis may be either flaccid or 
spastic; (3) the reflexes may be normal, deficient or increased; (4) a 
diminution of vision terminating in absolute blindness (the cherry-red 
spot in the region of the macula lutea and later optic nerve atrophy) ; 
(5) marasmus and a fatal termination, as a rule, before the age of two 
years; (6) the occurrence of the affection in several members of the 
same family. 

In some, but not in all of the cases, nystagmus, strabismus, and 
hyperacusis are added to the above cardinal symptoms. A loss of the 
sense of hearing was noted in two cases. Falkenheim refers to im- 
pulsive laughter, but I have not been able to persuade myself that this 
had occurred in any except in the one case described by him. Some 
slight variations will naturally occur in the degree and character of 
paralysis. The changes in the macula lutea are so striking that they 
constitute a very important symptom of the disease. But it is to be 
noted that the disease can be diagnosticated even in the absence of 
this one symptom, and that in some of the cases the general cerebral 
symptoms are developed some months prior to the retinal changes. 
Koller has reported a case in which at the first examination the changes 
in the macula lutea were not in evidence although they appeared later 
on, and in Higier's case the optic nerve atrophy was much more pro- 
nounced than the changes in the macula lutea. 

Etiology. — The causes underlying this disease are still obscure. In 
some of the families there has been blood relationship between the 
parents, in many others no such relationship existed. Injury to the 
mother during pregnancy has been noted in several of the cases and the 
tendency to mental derangement in the families of one or both parents 
is also suggestive of another possible factor. The family predisposition 
is evident from the fact that twenty-eight cases have occurred to my 
knowledge in fifteen families. Carter was the first to call attention to 
the fact that all of the cases reported have occurred among Hebrews, 
and even at this day I can say that I know of no indubitable case occur- 
ring in others. The racial feature of the disease is all the more astound- 
ing, because other diseases to which it must necessarily be more or less 
closely allied have been observed and recorded among all races and all 
nationalities. 

The absence of syphilis has been distinctly noted in most of the 
histories. This is a matter of some importance for there is always a 
tendency to attribute a mysterious family affection to this hereditary 
factor, and furthermore, the disease bears some resemblance to specific 
disorders which are characterized by various forms of dementia and by 
ocular disturbances (Batten). Starr was mistaken in quoting the 



AMAUROTIC FAMILY IDIOCY 535 

present writer as favoring the syphilitic etiology in this disease. In 
passing we may take note of the fact that there is an hereditary optic 
nerve atrophy occurring later in life which is due in many instances to 
hereditary syphilis, and with this optic nerve affection the present dis- 
ease might possibly be confounded. 

The disease runs its course, as was intimated above, in a little less 
than two years. I have encountered but a single prominent exception 
to this rule, and in this instance the child had attained the age of five 
and a half years when I examined it, and it is, so far as I know, still 
living. The characteristic symptoms of the malady were present, and 
the same symptoms had been presented by two or three other members 
of this family. Spiller threw out the hint that the symptoms and the 
anatomical changes may vary somewhat if some of these children should 
live beyond the usual term of years. Many of these children are ex- 
tremely well nourished at birth and for the first few months of life. 
By degrees they lapse into a state of marasmus to which they slowly 
succumb. I wish to insist on marasmus as a very important symptom 
of the condition, and the emaciation observed in such cases is so extreme 
that it unfortunately makes of the child a most disagreeable object. 
One writer has referred to sudden death as characteristic of the disease, 
but the very opposite of this has been observed in my experience. 

Pathological Anatomy — In my first case of palsy, in 18 87, the outer 
surface of the brain exhibited abnormalities which we were accustomed 
to associate with brains of inferior development. There was a con- 
fluence of the central and Sylvian fissures and a complete exposure of 
the island of Reil. There was unusual hardening, the knife grating 
on removing a small section of the cortex. On microscopical examina- 
tion the most important changes were found in the cortex, and sections 
were taken from the frontal lobes, motor areas, from the base of the 3d 
convolution, from the first temporal, and from a part of the apex. The 
same changes were found practically throughout the cortex. It was 
possible to make out the various layers of cells, but examination and a 
most careful search on removal of the sections showed that there were 
scarcely half a dozen pyramidal cells which presented anything like 
their normal appearance. The contour of the cells was either rounded 
or elongated, and the cell protoplasm exhibited every possible change 
such as we note in degenerated cells. In some cells the nucleus and 
necleolus were entirely wanting or were relegated to the margin of the 
cell. In later examinations further changes had been noted. Hirsch 
was the first to point out that these same cellular changes occurred not 
only in the gray matter of the cortex, but in the gray matter of the 



536 THE EYE AND NERVOUS SYSTEM 

entire central nervous system, in the cortex of the brain, in the basal 
ganglia, in the gray matter of the spinal cord, and even in the spinal 
ganglion. I was able to corroborate this finding by a later examination 
in another case of my own, and in 1903 I felt warranted in declaring 
amaurotic family idiocy to be a disease chiefly of the cortex and of the 
gray matter of the entire central nervous system. These findings have 
received further and most satisfactory corroboration in a careful report 
recently published by Spiller. Kingdon, Schaffer, and others, including 
Hirsch and myself, have found degeneration in the pyramidal tracts, 
yet this degeneration does not seem to be as marked as we would expect 
it to be from the very considerable involvement of the gray matter. 

Holden, Mohr, Treacher Collins, Schumway, and Buchanan have 
examined the eyes post-mortem. There is a degeneration of the ganglion 
cells of the retina, and of the nerve fibres of the optic nerves and tracts. 
There is some doubt as to the existence of an oedema in the region of 
the macula. 

The question has arisen whether the disease is invariably congenital, 
or whether it is an acquired disease. The latter is claimed to be the case 
by Hirsch, who would place it in the category of toxic affections ; but, 
as I have stated in several communications, it is hard to accept such a 
view for any family disease, and the fact that it cannot be attributed 
to the influence of the mother's milk is proved by the one circumstance 
that several of my patients were raised by strange wet nurses. There 
is no doubt in my own mind that the disease is developed as the result 
of a congenital defect. The nervous system of such a child is able to 
perform tolerably normal functions until the age of four or six months, 
then it is no longer able to meet the strain put upon it, and an active 
process of degeneration sets in. This degeneration is, to my thinking, 
a natural result of an arrest of development. Gowers has claimed very 
much the same for some of the scleroses that have developed very much 
later in life, and the fact that the first symptoms of amaurotic family 
idiocy become evident about six months after birth, does not militate 
against the theory of a congenital defective development. 

Amaurotic family idiocy should not be regarded as an isolated 
though interesting disease, but all the symptoms and anatomical findings 
suggest a relationship to other hereditary family affections. 

Treatment. — Unfortunately, little can be said in favor of any thera- 
peutic measures. In several cases under my observation I have hoped 
against hope that, by most careful nursing and feeding, some of the 
children thus afflicted might escape, but up to the present time all such 
efforts have been entirely fruitless. Kingdon has suggested the use of 



IXFAOTILE CEKEBKAL PALSIES 537 

thymus extract, but he is not yet able to state that any advantage has 
been gained thereby. It would be natural to infer that the wisest thing 
to do would be to avoid bringing such children into the world ; but, as 
several healthy children have been born to parents who have had one 
or two children afflicted with amaurotic family idiocy, advice bearing 
upon this point cannot readily be given. In conclusion I consider it 
necessary to remind the oculist that these patients can no longer be 
regarded as afflicted with a rare ocular condition, but that the ocular 
condition, interesting as it is, is merely one symptom of an easily 
recognizable family affection. 

INFANTILE CEREBRAL PALSIES (SPASTIC HEMIPLEC?IA, 
DIPLEGIA, AND PARAPLEGIA). 

Although varying widely in their mode of origin, the cerebral 
palsies of children deserve to be treated as a distinct clinical group of 
diseases. A study of the subject many years ago led me to divide them 
into three classes in accordance with the mode of onset, — viz., those that 
were prenatal in origin, those that were due to difficulties during parturi- 
tion, and those which were distinctly acquired some years after birth. 

Whatever the time or manner of origin may have been, most of 
these cerebral palsies are observed in the earlier years of life, — from the 
time of birth up to the age of ten years, even later; but by far the 
greater proportion of them occur during the first three years of life. The 
paralysis is of the distinctly spastic order and according to the distri- 
bution of the palsy we may distinguish between a hemiplegia and a 
diplegia (double hemiplegia), or paraplegia. Monoplegias are rela- 
tively rare, and some of those that appear to be monoplegic in character 
are practically the incomplete diplegias or hemiplegias. The rigidity 
of the muscles, the contractures resulting from the same causes, and in- 
crease of all the deep reflexes in the paralyzed members are the constant 
accompaniment of these palsies. Coma and convulsions may occur in 
the initial stage of the acute forms, and the convulsions may be often 
repeated during the later stages of the disease. The cerebral palsies 
of children are more frequently associated with coma and convulsions 
than are those of the adult ; the former are generally due to cortical 
processes, the latter to intra-cerebral conditions. To say that the con- 
vulsions may frequently be repeated is equivalent to saying that an 
epilepsy has been established which may continue throughout life, and 
the same changes which have caused both the paralysis and the epilepsy 
may also be responsible for the defective mental development, which 
may range from weakmindedness to marked imbecility and complete 



538 THE EYE AND KEKVOUS SYSTEM 

idiocy. It is especially to be noted that disturbances of motion, asso- 
ciated movements, ataxic, athetoid, choreiform, and even cataleptic 
movements, occur often enough in connection with these diseases. 

Aphasia, so common in apoplectic disorders of the adult, is a much 
rarer complication in infantile palsies. It goes without saying that all 
these conditions are characterized by certain negative symptoms which 
are of greatest importance in attempting a differential diagnosis be- 
tween the cerebral spastic and the spinal flaccid palsies. Such symp- 
toms include the entire absence of changes in the electrical reactions 
and the absence of any marked atrophy in association with the palsy, 
although a slight atrophy from disuse is common enough. 

Etiology. — In discussing the origin of cerebral palsies, we must bear 
in mind the three groups alluded to above. As for the prenatal cases, 
they may be due to hereditary taints, which is equivalent to saying that 
such cases occur in families with a decided history of various neuroses, 
with a history of hereditary epilepsy, or of hereditary insanity. The 
next most important etiological factor is unquestionably the occurrence 
of some traumatism to the mother during the period of pregnancy. The 
manner in which such traumatism may injure the brain of the child was 
clearly shown by Cotard, who found in the brain of a still-born child an 
old lesion in the right hemisphere in the vicinity of the lateral ventricle. 
Illness of the mother during pregnancy, exhausting fevers, particularly 
such as accompany pneumonia and typhoid fever, ursemic convulsions, 
and severe fright, are other causes which have been made out in cases 
of prenatal origin. 

My own experience would lead me to deny the influence of heredi- 
tary syphilis, but so many cases of this character have been described 
oy others that I must accept this as a possible etiological factor in some 
of the palsies. 

The etiology of a birth palsy is a very simple one. While the brain 
of the new-born child tolerates a surprising amount of pressure, it is 
natural that harm should occasionally be done. Asphyxia at birth, as 
Little was the first definitely to point out, has been considered a most 
potent factor. My own statistics have shown that tedious labor is a 
more frequent and a more disastrous factor than is instrumental de- 
livery. This explains why cerebral birth palsies occur with greatest 
frequency in first-born children. 

Acute cerebral palsies may be due to a variety of causes. They may 
come on after measles, scarlatina, typhoid fever, small-pox, and even 
tonsillitis. Their onset after pneumonia and whooping cough is very 
common, but in the case of the latter it is questionable whether the 



INFANTILE CEEEBKAL PALSIES 539 

palsy is due to the effect of the toxic agent or to the mechanical injury 
during a spasm of coughing. Fright, which Freud has dwelt upon, as 
the equivalent of a psychic trauma is an occasional cause of acute cere- 
bral paralysis, but actual injury to the skull is a much more powerful 
factor. These palsies occur also after a simple or cerebrospinal menin- 
gitis, after an exhausting gastroenteritis, and after other fevers. Of 
ninety-one cases of acquired cerebral palsy which I studied many years 
ago, the exact cause could not be ascertained in twenty-seven. 

There was a tendency some years ago, particularly among French 
writers, to claim that all cases of acquired cerebral palsy were due to 
acute infectious diseases. This view was also insisted upon by Strum- 
pell, who established the analogy between the acute cerebral and the 
acute spinal paralyses of children and concluded that the former are 
due to a polioencephalitis just as the latter are due to a poliomyelitis. 
While the dependance of acute or acquired cases of polioencephalitis has 
been very much over-stated, there is evidence enough to compel us to 
concede that some of these acquired cases may be due to an encephalitis. 
A discussion has arisen between other authors and myself regarding the 
etiological importance of convulsions. Freud and Rie are of the opin- 
ion that convulsions denote the onset of the cerebral process, but that 
they never hold a causal relation to the palsy which results from the 
cerebral lesion. While one may be willing to grant that in a very large 
number of cases this statement of the Austrian authors holds good, 
Osier and myself have advanced the view that in many others the palsy 
seems to be more or less the immediate result of the convulsive seizure. 
Anyone who has observed the marked disturbances of circulation at the 
acme of a convulsion, can readily conceive how easily a blood-vessel 
could burst during this period as a result of excessive stasis ; and that 
such venous hemorrhages occur in epileptic convulsions I have had rea- 
son to know from the examination of the brain of a girl of seventeen 
years, who died three days after a severe epileptic attack. In this case 
there was a large subpial extravasation, which covered quite completely 
the entire left hemisphere and part of the right. If such an occurrence 
could take place in the brain of a girl of seventeen, there is no reason 
why similar accidents of lesser extent should not happen frequently 
in the case of children. There is also further clinical evidence on this 
point, and those who are specially interested in this subject are referred 
to a discussion of it in my book on " Nervous Diseases of Children," 
page 531. 

Symptomatology. — The distribution of the paralysis may vary with 
the condition of the paralyzed limb and is the same in all cases. The 



540 THE EYE AND NERVOUS SYSTEM 

hemiplegia form resembles in every respect the hemiplegia of the adult, 
and further analogy is observed in this that the leg recovers very much 
more* quickly than the arm, just as in adult hemiplegia. Under 
diplegia, we may classify all those cases in which both halves of the 
body have been involved, and it is better to attach the greatest impor- 
tance to this bilateral character of the palsy even though the palsy be 
incomplete, for the bilateral affection points to a double brain lesion 
and that is, after all, the salient point for consideration. In diplegias, 
the legs may be much more affected than the arms, there may be cross- 
legged position, and cross-legged progression, and in some instances the 
arms may appear to have escaped to such an extent that what is an in- 
complete diplegia may appear to be a paraplegia, but a paraplegia asso- 
ciated with athetoid or other disturbances of motion in the upper ex- 
tremities could be designated as an incomplete diplegia ; but whether the 
form of paralysis be a diplegia or a paraplegia, such paralysis must 
necessarily be due to a double cerebral lesion and these are almost in- 
variably prenatal in origin or the result of traumatism during labor, 
and yet it is well to state that both diplegias and paraplegias also occur 
every now and then in the acute cerebral cases, contrary to the opinion 
of Strumpell, who holds that infantile hemiplegia is synonymous with 
the acute cerebral paralysis of children. 

Aphasia may be associated with these infantile cerebral palsies. 
It is invariably motor, never sensory, in character. It need not be 
added that aphasia will be developed only in children who have acquired 
articulate speech before the onset of the paralysis. This excludes from 
the list all diplegias and paraplegias which have come on before or 
during birth. It must be remembered that a complete absence of speech 
development may be present in children who exhibit cerebral and mental 
defects and I have seen some children in whom the mental defect seemed 
to be due more largely to defective development of the speech centres 
than of any other part of the brain. 

In addition to the form of paralysis, it is well to pay special atten- 
tion to the rigidities and contractures. These abnormal conditions of the 
muscles are so prominent that doubt has arisen whether some of the 
diplegias or paraplegias would not be more properly spoken of as cases 
of congenital spastic rigidity, for the rigidity is most marked and the 
paralysis often very slight. In the case of Cotard quoted above in 
which the palsy was due to an intrauterine lesion, the child was born 
with contractured extremities. The contractures may vary in degree 
and in the number of joints affected. The flexors and pronators of the 
arms, flexors of the legs and of the feet are most frequently involved. 



INFANTILE CEKEBKAL PALSIES 541 

In the case of diplegia and paraplegia there is, in addition, contracture 
of the adductors of the thighs, which is responsible for the peculiar 
cross-legged progression and cross-legged position. Pes equinus or pes 
equino-varus is the most common deformity of the foot. In a few cases 
an equino-valgus is present. If the upper extremity is contractured the 
arm is held in close juxtaposition to the trunk, is flexed at the elbow, 
and the hand is generally in a position of extreme flexion, the fingers 
often being firmly pressed into the palm of the hand. 

The post-paralytic disturbances of motion constitute a very charac- 
teristic group of symptoms in cases of infantile cerebral paralysis. 
While they are seen occasionally in adult apoplexy, they are far more 
common in the paralytic disturbances of youth. The choreiform move- 
ments are of importance because cases of that kind are often mistaken 
for cases of ordinary chorea, but of course the presence of paralysis or of 
contractions, and the exaggeration of the reflexes in the choreic ex- 
tremity, will be sufficient to indicate the nature of the choreic move- 
ments. Athetoid movements are of special interest, inasmuch as they 
are observed invariably in connection with cerebral palsies. Associated 
movements are often developed to a marked degree, the paralyzed hand 
imitating all movements of the normal hand. 

Epilepsy is, perhaps, the most serious of all conditions associated 
with infantile cerebral paralysis. The special point of importance is 
that the palsy may disappear or may be so slight as to give little in- 
convenience to the child, whereas the epilepsy remains. Special reason 
for insisting upon this point is that in such cases the epilepsy as well as 
the paralysis is of organic origin and we cannot therefore expect to cure 
the disease by the use of the ordinary measures. 

Idiocy is also developed very often both in association with epilepsy 
and with cerebral palsies. The severer forms of mental retardation are 
present in cases of diplegia and in cases of paraplegia rather than hemi- 
plegia, and this association can be explained easily enough if we remem- 
ber that the diplegias and paraplegias point to a double lesion, whereas 
the hemiplegia results from a lesion in only one-half of the brain. 

As for the eye symptoms observed in these conditions, the com- 
monest are strabismus and occasionally nystagmus, and as Freud has 
pointed out, hemianopsia may be associated with the brain palsies of 
children. The latter condition is surely a very uncommon one and 
would depend largely upon the accidental involvement of the occipital 
region in a case of cerebral defect or in a case in which considerable 
blood had been exuded over the occipital region of the brain during 
parturition. The pupillary reflexes are normal in such a vast majority 



542 



THE EYE AND NERVOUS SYSTEM 



of these children that they are of no symptomatic value. Freud has 
reported some cases of optic atrophy associated with diplegias and he 
thinks that these conditions, which are rare enough, may bear a very 
close relation to the changes found in cases of amaurotic family idiocy. 
I have never seen changes in the macula lutea in any case of distinct 
cerebral spastic paralysis. 

Morbid Anatomy. — The entire subject of morbid anatomy of infantile 
cerebral palsies will be sufficiently stated in this connection by the repro- 
duction of a table which was first embodied in the chapter on these 
diseases in the author's text-book, and which has been very fully quoted 
by later authors. I have nothing to add to it, and believe that it fully 
represents our present knowledge of the subject. 

CLASSIFICATION OF INFANTILE CEREBRAL PALSIES. 



GROUPS. 



I. Paralyses of intra-uterine onset. 



II. Birth palsies 



III. Acute (acquired) palsies 



MORBID LESIONS. 

Large cerebral defects (porencephaly). 

Defective development of pyramidal 
tracts. 

Agenesis corticalis (highest nerve ele- 
ments involved ) . 

Meningeal hemorrhage, rarely intra- 
cerebral hemorrhage. Later condi- 
tions: meningo-encephalitis chronica;, 
sclerosis, and cysts; partial atrophies. 

Hemorrhage (meningeal and rarely in- 
tracerebral ) ; thrombosis from syph- 
ilitic endarteritis and in marantic 
conditions: atrophy, cysts, and scle- 
rosis ( diffuse and lobar ) . 

Meningitis chronica. 

Hydrocephalus (seldom the sole cause). 

Primary encephalitis; polioencephalitis 
acuta ( Strumpell ) . 



Differential Diagnosis- — First of all it is important to distinguish 
between the cerebral and the spinal paralyses of children, but anyone 
who is able to recognize the difference between brain and spinal lesions 
will be able to make this differential diagnosis. In the one case we have 
spastic paralysis, increased reflexes, normal electrical reactions, no 
atrophy, and generally a hemiplegic or diplegic form of paralysis. In 
the spinal group the paralysis is flaccid, the knee-jerks are absent, there 
is very marked atrophy and the paralysis may affect several members 
of the body, or several large groups of muscles, but is never hemiplegic. 
Moreover, the electrical reactions in the spinal cases are always in- 
volved and altered. Difficulties may, however, arise in some mild cases 



INFANTILE CEKEBKAL PALSIES 543 

of cerebral paralysis in which the spastic contractions are but slightly 
developed and the reflexes not very much increased. The presence of 
athetoid or choreic movements in one-half of the body points to cerebral 
paralysis, but these are never associated with spinal palsies. 

The oculist should bear in mind that the nystagmus and strabismus 
may be symptoms of multiple sclerosis, but the differential diagnosis 
should be based upon scanning speech and upon the absence of associated 
movements, and the post-paralytic disturbances of motion which are so 
common in the infantile spastic palsies. Inasmuch as there is a heredi- 
tary form of multiple sclerosis this differential diagnosis may have to 
be made with the greatest possible care. A very distinct hemiplegia or 
a diplegia would militate against the diagnosis of multiple sclerosis. 

Treatment. — The treatment of infantile cerebral palsies calls for 
deliberate non-interference at the start. The treatment of the later 
stages will be practically the same whether the case be congenital or 
acquired. If a young infant exhibits a tendency to drowsiness or to 
convulsions, its brain has in all probability sustained a serious injury 
during labor. Strict attention should be paid to the nutrition of the 
child, and milk properly prepared according to the age of the child, or 
else breast milk, is the only food to be administered. If there is a very 
marked tendency to convulsions, small doses of bromide or minimum 
doses of morphia or chloral should be given. If convulsions continue in 
spite of these measures, careful inhalation of chloroform may be prac- 
tised. All other measures, as counter-irritation, mustard baths and the 
like, are useless. 

In a case of an acute cerebral palsy in a child, the fewer drugs that 
are administered the better. The physician will do well to limit his 
therapeutic efforts to the administration of calomel in doses sufficient 
to procure a free purging of the bowels, and small doses of bromides to 
secure rest for the disturbed brain. At a later period bromides may be 
combined with small doses of iodide, but whenever nutrition is dis- 
turbed, it is best to abandon all drug treatment. After the symptoms of 
the initial period have passed, the physician is called upon to prescribe 
some form of treatment for the paralysis and the other symptoms of the 
disease. After the paralytic condition of the active period of the 
disease has passed, the condition is to be treated by massage and elec- 
tricity applied to the paralyzed parts, but let the brain severely alone. 
While electricity applied to the extremities can do no harm, the effect on 
the injured brain is altogether too uncertain. When epilepsy is devel- 
oped the condition must be treated as all other organic epilepsies should 
be and in some of these cases, according to the experience of Horsier. 



544 THE EYE AND NERVOUS SYSTEM 

Wyeth, Gerster, and myself, there is sufficient reason to attempt surgical 
interference, particularly if all the symptoms point to a well localized 
lesion, and above all, if there is a probability of the existence of an old 
hemorrhagic cyst, a condition which is found in not a few of the con- 
genital and acute cases. In children in whom the rigidity is the most 
marked symptom, therapeutic measures are in order and tenotomies, as 
well as transplantation of tendons, yield good results in the hands of 
competent men. 

BIBLIOGRAPHY. 

(For additional references, see author's article in Journal of Nervous and Mental 

Disease, January, 1903.) 
Barter: Archives of Ophthalmology and Otology, 1894. 

2 Claiborne: Archives of Pediatrics, 1900, vol. x. p. 3. 

3 Collier: Brain, 1899, vol. xxii. p. 373. 

4 Freud: Zur Kenntniss der Cerebralen Diplegien des Kindesalters, Vienna, 1893; 

Die Infantile Cerebrallahmung, Vienna, 1897. 

5 Falkenheim : Jahrbuch f. Kinderheilkunde, 1901, N. Y., vol. liv. 

6 Frost: Fundus Oculi, Edinburgh, 1896. 
7 Goldzieher: Hirschberg's Centralblatt, 1885, S. 219. 
8 Heiman: Archives of Pediatrics, April, 1897. 

9 Higier : Zur Klinik der Familiaren Opticusaff ectionen ; Zeitschr. f . Nervenheil- 

kunde, 1897, Bd. x. 
10 Hirschberg : Centralblatt f. Augenheilkunde, 1888. 

"Hirsch, W.: Journal of Nervous and Mental Disease, 1898, vol. xxv. p. 538. 
^Kingdon: Transactions of the Ophthalmological Society, Heidelberg, 1895; with 

Russell, Medico-Chirurgical Transactions, 1897, vol. lxxx. 
13 Koller: New York Medical Record, 1896. 
14 Koplik: Archives of Pediatrics, 1897. 
"Magnus: Zehender's klinischer Monatsblatter, 1885, Bd. xxiii. S. 357. 

16 Muratow : Zeitschr. f . Nervenheilkunde, Bd. x. S. 234. 

17 Pelizaeus : Archiv f . Psychiatrie, Bd. xvi. S. 698. 

18 Sachs, B.: Journal of Nervous and Mental Disease, 1887, 1892, and 1903; Volk- 

mann's Vortrage, 1892, Nos. 46, 47; Nervous Diseases of Children, p. 396; 
New York Medical Journal, May 30, 1896. 
19 Spiller: Journal of Nervous and Mental Disease, 1901, p. 140; American Journal 
of the Medical Sciences, January, 1905. 

20 Tay, Waren : Transactions of the Ophthalmological Society of the United King- 

dom, vols. i. and iv. 

21 Wadsworth : Transactions of the American Ophthalmological Society, 1887. 



CHAPTER XIY. 

ABNORMALITIES US THE DEVELOPMENT OF THE BRAIN 
AND SKULL j FACIAL HEMIATROPHY ; FACIAL HEMI- 
HYPERTROPHY ; ACROMEGALY. 

By CHARLES W. BURR, M. D. 

I shall riot consider anencephaly or any of the conditions in which 
the abnormality of development or the intrauterine disease is so great 
as necessarily to cause fetal death. Such conditions are of great interest 
in the study of the causation of monsters, but are of no clinical impor- 
tance. The normal development of the brain and that of the skull are 
closely associated with each other and pathological develoj^ment in either 
may affect the other, but for convenience sake they are studied sepa- 
rately. Strictly speaking, the term " defects of development" should be 
used to mean only defects due to an improper growth of the brain on 
account of inherent deficiency in the germ or sperm cell and should 
not include abnormalities caused by acquired disease occurring in the 
embryo ; but our ignorance of embryonal pathology is so great that such 
a distinction can rarely be made. 

Apart from the abnormal development which causes gross defects 
there is another kind of lack of development, really congenital in origin 
though the symptoms of disease do not appear till some time, it may 
be years, after birth. I mean that condition in which the patient may 
at birth seem normal, may indeed pass normally through infancy, and 
then, or even later, show symptoms of disturbance of the functions of 
the brain or cord, not produced by any of the known causes of disease, 
but by inherent weakness and inability to withstand the physiological 
stress of life. This weakness may exist not only in the nervous system 
but in any part of the body. It has been recently described, or rather 
offered as an explanation (for, of course, it is a theory and not an estab- 
lished fact), by Sir William Gowers under the title '-'Abiotrophy." 
.He states, what has been long known, that life is a double thing, — 
somatic, that of the body as a whole, and local, that of each element by 
itself. Death of some of the elements may occur without somatic life 
being affected. This process we call degeneration, but it is really a 
slow decay, a local death due to an essential failure of vitality. This 
process may affect not only some one system of nerve-fibres in the brain 
35 545 



546 THE EYE AKD ^ T EEVOUS SYSTEM 

or cord, as in Friedreich's ataxia or congenital cerebellar atrophy, but 
also the muscles, as in the various forms of idiopathic muscular atrophy. 
Gowers believes that there is a certain type of degeneration of the optic 
nerve, occurring in families, which is really abiotic, really due to an 
inherent weakness of the nerve-fibres and in which tobacco or some other 
poison acts only as an exciting, not a true, cause. This theory does not 
claim that all degeneration in the pathologist's sense of the word is 
abiotic. 

Porencephaly. — The word porencephaly was first used by Herschl 
and the first elaborate monograph was written in 1882 by Kundrat. By 
them it was used to mean a hole going into the brain, not the entire 
absence of brain structure and not minute cavities resulting from small 
vascular lesions. Porencephaly is classified as congenital, coming on 
during intrauterine life, or acquired, occurring at or very soon after 
birth. Clinically the differentiation is always difficult, often impossible. 
Practically it does not matter to which type a case belongs. The con- 
genital cases like the acquired are probably always due to disease and 
not the result of a true developmental defect. 

The cavity may occur in any part of the brain but is most frequent 
in the anterior or middle. Usually there is only one cavity but there 
may be two situated in the same position in either hemisphere. For 
example, the parietal region may be affected on both sides or both 
frontal lobes. When there is only one cavity it is more apt to be in 
the right hemisphere. Brill states that no case has been reported in- 
volving the median surface of the hemisphere, but Brush reports a case 
in which the cuneus was much affected. 

Kundrat holds that the immediate cause is anaemic necrosis. 
Schultze would sharply separate the cases of uterine origin from those 
occurring after birth by the following rule: In the former, he states, 
the convolutions are so arranged as to radiate toward the margin of the 
cavity or towards its centre, while in the latter the hole is irregular in 
outline and the convolutions have no regular arrangement. The most 
frequent direct cause is probably vascular or meningeal inflammation. 
Traumatic encephalitis may occur during intrauterine life and cause 
the formation of a cavity. Schultze, for example, holds that spasmodic 
contraction of the uterus arising from causes within itself may injure 
the fetal brain. Alcoholism in the mother and syphilis in either parent 
have been cited as causes of obliterating arteritis in the foetus. B. 
Sachs speaks of the possibility of some purulent disease in the mother 
during pregnancy as being sufficient to cause meningitis or encephalitis 
which might, occurring in a growing foetus, produce porencephaly. In 



ANOMALIES OF BRAIN AND SKULL 547 

acquired porencephaly the primary lesion seems almost always to be a 
meningeal hemorrhage occurring at birth and resulting from pressure on 
the skull during labor. Whether meningeal hemorrhage occurs most 
frequently from the use or non-use of the obstetric forceps is much dis- 
cussed. Probably more injury has been done by the non-use of the 
forceps than by their unskilful application. Whatever may cause the 
hemorrhage, if it be large enough, there is compression of the brain 
substance, atrophy of the cortex and underlying white matter and a 
consequent cavity. The pia and cortex are apt to become inflamed, 
agglutinate, form a dense membrane lining the cavity which is filled 
with fluid, thus forming a cyst covered by the outer membranes. The 
time required for the absorption of the brain tissue and the formation 
of the cavity is unknown. Acquired porencephaly is most apt to occur 
in the parietal region and is usually unilateral, but may be bilateral. 

There are no pathognomonic symptoms. It is not difficult to deter- 
mine the existence of organic brain disease, but it is impossible to say 
with certainty whether the symptoms are due to porencephaly or to 
cerebral atrophy without cavity formation. Almost always, but not 
invariably, there is marked mental defect. Usually there is idiocy or 
at least imbecility. Rarely in unilateral cases without great destruction 
of the cerebral tissue the mind may be unimpaired. The most frequent 
physical symptoms are spastic hemiplegia or diplegia. Epileptiform 
convulsions are very frequent. If the cuneus is involved hemianopsia 
must result. Often there is a spastic disorder of speech and sometimes 
a true aphasia or rather, from the destruction of the potential speech 
centres, an inability to learn to speak. Probably the majority of the 
patients, certainly a very large number, die in early life ; a few survive 
to maturity, and even old age. 

Microcephalus. — The term is used to mean only those cases of a 
small brain associated with a small skull and due to defective develop- 
ment of the entire organ. It must not be forgotten that a skull of 
normal size may contain a brain of very small volume, the surplus 
space being filled by fluid. Any skull under seventeen inches in cir- 
cumference is, according to Dr. Ireland, to be classed as microcephalic. 
The head is, as a rule, cone-shaped or oxycephalic. The forehead slants 
backward in marked manner. The palate is generally flat and the base 
of the skull asymmetrical. The face is never proportionally as small 
as the cranium. The causes of the condition are very obscure. There 
is no doubt that a few cases are produced mechanically by a pre- 
mature ossification of the sutures preventing the growth of the brain. 
This was Virchow's theory. Klebs's theory that hour-glass contrac- 



548 THE EYE AND NERVOUS SYSTEM 

tion of the uterus on the fetaT head is a cause is no longer regarded 
with favor. It is probable that some cases are caused by intrauterine 
cerebral inflammation. B. Sachs describes cases occurring after birth 
and following convulsions in which it was probable, he thinks, that the 
convulsions resulted in extensive meningeal hemorrhage or pachy- 
meningitis hemorrhagica, in consequence of which there was atrophy 
of the brain and associated with it a lessening in the growth of the 
skull. In these cases the children were normal at birth and with skulls 
of average circumference. The relation, under normal conditions, be- 
tween the growth and development of the brain and skull is very 
obscure. Since, however, there may be a small and universally diseased 
brain associated with a skull of normal size, the space being filled with 
fluid, it is not probable that the brain exerts any trophic influence on 
skull growth. 

Though the entire brain is affected in microcephaly the cerebral 
hemispheres are most deficient. The cerebellum and the basal ganglia 
are less abnormal. The spinal cord, as a rule, is also shorter and 
smaller than normal. The columns of Goll, the anterior horns, and 
pyramidal tracts are especially affected. Sometimes even with spastic 
diplegia the cord is only small and shows no degeneration. Small as 
the brain may be, and Ireland reports one the weight of which was 
seven ounces, it is distinctly human in form, and could never be mis- 
taken for that of one of the lower animals. The primary fissures are 
always present, the secondary may be absent or may be replaced by 
others much more numerous, making a large number of small convolu- 
tions (microgyria). In old times the normal brain was compared in 
appearance to a bunch of worms; the resemblance is much greater in 
some cases of microcephaly. In the cases in which there are fewer 
fissures than normal the surface of the brain is, of course, smooth. 
It is of historic interest that at the time when Darwinism was a new 
thing, Charles Vogt wrote a book to prove that microcephaly was atavis- 
tic, — was a reappearance of the simian type. This opinion has not 
stood the test of time. 

The brain is deficient not only macroscopically but also microscop- 
ically. There are fewer nerve-cells than normal in the cortex and in the 
anterior horns of the spinal cord, and many of the cells present in the 
cortex either have no processes or greatly stunted ones. In some cases 
there is an overgrowth of connective tissue. 

The condition is not common; indeed it ranks among the rarer 
forms of idiocy. It can, of course, be diagnosed at sight, but except in 
the cases of extreme smallness of the head, it is not always possible to 



ANOMALIES OF BRAIN AND SKULL 549 

predicate the extent of idiocy from the appearance of the head. In 
other words, barring the extremes, the idiot with the smaller head may 
have more intelligence than he who has the larger. The explanation 
of this is, that in the former case the cortical cells themselves are less 
diseased. There may be no physical symptoms beyond a general dwarf- 
ing of the entire body ; on the other hand the patient may not be 
able to stand or walk, feed himself, speak, or indeed do anything save 
exist if cared for. Those of a higher grade are active, energetic, easily 
made angry, affectionate but wilful, having no power of continnons 
attention, — indeed resembling superficially, but only superficially, the 
monkey tribe. As a rule, they either cannot speak at all or only a few 
words. This is probably not a true aphasia, but due to their general 
low mental state. They are apt to be thieves, but to appropriate things 
useless or useful indiscriminately. Fortunately, the sexual instinct in 
the majority of cases is wanting. In some the organs of generation 
develop, but I know of only one case, that of Ireland, in which a micro- 
cephalic woman became pregnant. The child was born dead. 

There is no treatment save to give what little education is possible. 
The surgical intervention (craniectomy) proposed by Lannelongue has 
fallen into disuse. It was based on a false pathology and had no 
good results. 

Local Cerebral Defects. — As said above, a skull of normal size 
may contain an abnormally small brain. Besides this there may be an 
atrophy or a lack of development of some one part of the brain, — for 
example, one cerebral hemisphere. Most frequently this is caused by 
hemorrhage in the meninges at birth, but the disease process may occur 
during intrauterine life. Osier, for example, reports the case of a 
foetus about six months old, in the left basal ganglia of which was a 
recent clot which had broken into the ventricle. The mother had died 
of typhoid fever. It is scarcely possible that any foetus could survive 
till birth after suffering a cerebral hemorrhage, but it is very possible 
that many of these cases are caused by intrauterine obliterating vascu- 
lar disease causing lack of growth. Whether any of them are really 
developmental defects is not yet determined. Clinically, atrophy of 
one hemisphere is a frequent cause of a congenital spastic hemiplegia. 
The paralyzed side is the seat of choreic or athetoid movements. Epilep- 
tiform convulsions are frequent. Frequently there is imbecility. Mills 
relates a case, which was not, however, intrauterine in origin, but 
followed convulsions at eighteen months, in which mirror-writing was 

O 7 © 

present. This is met with now and again in both the intrauterine 
and birth hemiplegias. Hemianopsia or irregular contraction of the field 



550 THE EYE AND NERVOUS SYSTEM 

of vision may occur. There is often no visual disturbance. In many 
of the cases in which the atrophy occurs during intrauterine life the 
skull is very asymmetrical, being of much smaller volume on the dis- 
eased side. This was so marked in one patient on whom a surgeon 
operated that the longitudinal sinus was displaced and he cut into it, 
causing an outflow of blood, and death. 

There are still more localized defects of development or diseased 
areas. Thus the frontal lobes may be defective and the child, pre- 
senting no paralytic or sensory symptoms, will be mentally deficient. 
Peterson reports a case in which both occipital lobes and the motor 
area on the right side were atrophied. The patient was idiotic, epilep- 
tic, slightly hemiplegic, and could scarcely distinguish light from 
darkness. 

Some cases of congenital cerebellar ataxia seem to be due to real 
defects in development, not to disease but to an error in development 
inherent in the embryo. Thus, Nonne describes a small cerebellum, 
without sclerosis and without degeneration, indeed normal in all regards 
except as to relative size. The spinal cord was also diminutive. Before 
Nonne, Marie had described a similar condition under the title " heredi- 
tary cerebellar ataxia." The symptoms are somewhat like those of 
Friedreich's ataxia. There is titubation, choreiform or ataxiform move- 
ments of the arms and legs, and increased knee-jerks. The ocular symp- 
toms are important. There is nystagmus, diplopia, and sometimes 
amblyopia or color-blindness, or contraction of the visual fields. Optic 
nerve atrophy is characteristic. Other authors have described cases 
more or less resembling this type, and more or less departing from it. 
Indeed, there is clinically an unbroken chain of cases from Friedreich's 
ataxia on the one side to the so-called hereditary spastic palsy on the 
other. The difference in symptomatology must depend upon which 
part of the cerebrospinal axis is lacking in development. If the cere- 
bellum alone be deficient the symptoms can not be the same as when 
the cord alone or both cord and cerebellum are involved. The symp- 
toms of hereditary ataxia do not appear at birth but later, usually at 
about puberty. 

Absence of the Corpus Callosum. — Nothing is definitely known of 
the functions of the corpus callosum except that it contains commissural 
fibres binding together the hemispheres. Bruce 1 states that it may be 
entirely absent without the patient showing any symptoms. As a rule, 
however, its absence is associated with other cerebral defects and accom- 
panied by idiocy or imbecility. 

1 Brain, vol. xii. 



ANOMALIES OF BRAIN AND SKULL 551 

Abnormal Cerebral Fissuration. — Slight variation in the direction 
and number of the minor fissures of the brain is common. It has no 
pathological significance. Sometimes the variation from the normal 
is very marked and it has been attempted to set up a type of criminal, 
epileptic, and congenitally insane brain. , Moritz Benedikt studied the 
matter for years and concluded that in either of these conditions certain 
cerebral stigmata are present. These are excessive development of prin- 
cipal and subordinate fissures and excessive confluence of fissures either 
by elongation of the principal fissures, or by their anastomoses through 
the great development of subordinate fissures connecting them. Bene- 
dikt's papers led to much discussion, which indeed still continues, and 
the question remains unanswered whether there is a type of brain which 
predestines its owner fo become a criminal or an epileptic. In at least 
one form of insanity congenital in origin, — namely, paranoia, — there 
are sometimes marked deviations from the normal fissuration. Berkley 
says that in his experience it is the most striking thing on gross examina- 
tion of the brain in that disease. Really no other gross lesion is ever 
present. 

Cerebral Heterotopia. — Occasionally there is found on microscopic 
examination of a brain, usually one showing gross defects or abnormali- 
ties, small gray nodules or streaks in the white matter of the cerebrum 
or cerebellum due to the presence of tissue rich in nerve-cells and poor 
in medullated fibres. They are bits of gray matter out of place. Van 
Gieson has shown that in the spinal cord heterotopia is frequently an 
artefact, occurring while the cord is being hardened or cut and mounted. 
This is not always the case, however. Misplacement of tissue may 
occur in the brain just as it does in the abdominal organs. Its cause 
is not clear. It probably, however, is caused either by an abnormal 
development of nerve-fibres causing the detachment of a small bit of 
gray matter from its proper place, or by the primary development of 
gray matter in such a situation as to be later surrounded by white 
matter. Occasionally also anomalous bundles of white fibres are found 
in the medulla. Heterotopia never produces any symptoms. 

Congenital Hydrocephalus. — External hydrocephalus is an accu- 
mulation of cerebrospinal fluid within the subarachnoid space. It is 
frequent in cerebral atrophy. In internal hydrocephalus the fluid is 
within the ventricles. It is caused by developmental defect of struc- 
ture, intrauterine inflammation of the ependyma, and pathological con- 
ditions of the blood. It may occur in children of healthy parentage 
and whose mothers have passed through pregnancy without any illness. 
On the other hand, several children in a family mav be affected. As a 



552 THE EYE AND NEKVOUS SYSTEM 

rule, but not always, the head shows enlargement at birth. Many chil- 
dren with this disease are born dead, some survive for a short time, a 
few, and if very slight cases are included, a quite large 'number, attain 
full maturity. The shape of the head is characteristic : the cranium is 
hemispherical or elipsoidal and the face appears relatively small. The 
bones of the vault are thin and separated. Many Wormian bones are 
present. The circumference of the skull varies greatly. It has been 
as much as forty inches. The amount of fluid varies greatly. The 
lateral ventricles are, of course, always dilated and their walls may 
be as thin as paper, the upper parts of the hemispheres being mere sacs. 
The cortex resists the pressure better than the white matter, but it, too, 
in a case at all severe, is decreased in thickness. The membranes, as a 
rule, are normal or slightly thinned. If the cause be inflammatory the 
choroid plexuses and the membrane lining the ventricles are thickened. 
The f ontanelles bulge and pulsate . with the heart-beat. If the child 
survive, many nervous symptoms develop. The mental symptoms vary 
from idiocy to high-grade imbecility. Some very slight cases present 
no symptoms at all except the deformity of the skull. Often there are 
cerebral palsies and epileptiform convulsions. There may be total 
blindness from atrophy of the optic nerves produced by direct pressure 
upon the chiasm and tracts. Optic neuritis is much less common. 
Strabismus is frequent. The diagnosis presents no difficulty. Treat- 
ment, either medical or surgical, has so far availed nothing. 

Defective Development of the Cranial Nerve Nuclei. — Glowers 
describes under the title " infantile oculofacial palsy" a rare condi- 
tion which may be present from birth or appear later, characterized by 
palsy of the external ocular muscles, paralysis of the face, and ptosis, 
but without involvement of the internal ocular muscles. He suggests 
that it is due to defective vital endurance in the nuclear structures. 
Sachs, using Schapringer's case as a text, shows that the motor branch 
of the fifth, the hypoglossal, the third, the sixth and seventh nerve 
nuclei may all be affected. On the other hand, only the nucleus of 
one facial nerve may be affected. I know of no post-mortem study of 
this condition, but it would seem to be caused by a true defect in 
development. 

Congenital Absence of the Visual System. — This is a very rare 
condition. Absence of the visual system within the skull never occurs 
save in association with anophthalmos or microphthalmos. It is dim- 
cult to tell where to draw the line separating these two conditions. E. 
Treacher Collins and J. H. Parsons lay down the following rule: 
When there is complete failure of the essential nervous mechanism of 



ANOMALIES OF BKAIN AND SKULL 553 

the eyeball, anophthalmos is the proper term ; when any of the nervous 
elements are present, no matter how imperfect, microphthalmos. Total 
absence of the eyeball is probably always a true developmental defect : 
microphthalmos is probably sometimes developmental, sometimes due 
to intrauterine disease. Zentmayer and Goldberg 2 describe very care- 
fully the microscopic appearances in a case of microphthalmos. More 
interesting to neurologists, however, are the studies which have been 
made of absence of the visual system within the cranium. Quite a 
number of cases have been studied, but that of Spiller 3 is the best, and 
I shall quote largely from it. The patient was an idiot boy twenty-two 
years old. Spiller had regarded him in life as a case of cerebral spastic 
paraplegia with absence of the eyeballs. At autopsy the optic foramina 
were found to be absent. The orbital contents were not allowed to be 
removed and hence whether there was a rudimentary eye present could 
not be determined. Nothing resembling an eyeball was seen. No trace 
of optic nerves, chiasm, or optic tracts could be found. There was no 
sign of an external geniculate body on either side, and the thalamus 
on each side had nothing resembling an optic tract passing to it. 
The brain was small, firm, and not oedematous. The left ascending 
frontal convolution in the centre for the arm was very small. The occip- 
ital lobes and the cunei were very small. The calcarine fissure was 
short. The external arcuate fibres were unusually well developed. 
The spinal cord was small, but otherwise normal. On microscopic 
examination the cortex of the left calcarine fissure possibly contained 
fewer cells than normal. The optic radiations in the frontal sections of 
the occipital lobe were not entirely absent, Spiller draws the following 
conclusions : The chief " primary" optic centre is the external genicu- 
late body. The pulvinar of the optic thalamus is also an important 
" primary" optic centre. The anterior colliculus of the quadrigeminal 
body in man has an unimportant relation to vision. The hypothalmic 
body, the habenula, and the internal geniculate body, probably are not 
parts of the visual system. The cortex of the calcarine fissure may con- 
tain nearly the normal number of cell bodies, even though the visual sys- 
tem may be undeveloped. The nerves to the ocular muscles and their 
nuclei may be developed, even though the visual system is absent. A 
condition such as this (See Chapter I, Tigs. 1 to 4) is developmental 
and not caused by acquired disease, but as to the cause and mechanism 
of the developmental defect we know nothing. Nothing is known with 



Annals of Ophthalmology, January, 1904. 

University of Pennsylvania Medical Bulletin, February, 1902. 



554 THE EYE AND NERVOUS SYSTEM 

any certainty as to any hereditary influence which might act as a pre- 
disposing canse. All the cases I know of, have occurred in people with 
more or less mental deficiency. It is sometimes associated with cleft 
palate or harelip. The majority of the patients die immediately or not 
very long after birth. Only a few reach maturity. 

Abnormalities in the Development of the Skull. — I shall consider 
only the defects of growth which cause congenital cranial hernia and 
the abnormalities which are associated with certain ocular disturb- 
ances. The microcephalic skull has already been described. Cranial 
hernia includes encephalocele (protrusion of the brain substance), 
hydrencephalocele (encephalocele containing fluid and with a cavity 
usually communicating with the ventricles), and meningocele (pro- 
trusion of the cerebral membranes). 

Almost always the hernia occurs between bones and not through a 
hole in a bone. It almost always occurs in the median line, usually in 
the occipital, rarely in the frontal region. It very rarely is seen 
between the temporal and parietal bones. Cranial hernia produces a 
tumor under the scalp. An encephalocele is usually opaque and with 
a broad base. Meningocele and hydrencephalocele are translucent and 
23edunculated. It is justifiable to puncture with a hypodermic needle 
to determine whether the tumor is solid or contains cerebrospinal fluid. 
If the latter be present the tumor must be intracranial in origin and 
cannot be a vascular growth, a sebaceous cyst, or an abscess. All of 
these have been mistaken for cranial hernia. Frequently the opening 
in the bone can be felt, the mass may pulsate with the heart beat, and 
may increase in size as the child cries. Various other anatomic defects 
are apt to be present. Convulsions often occur. As a rule, the patients 
die after a very short time. In meningocele the sac has been success- 
fully removed. 

Congenital Deformity of the Skull associated with Optic Atrophy. 
— Post-neuritic optic atrophy associated with deformity of the skull 
has been reported in a small number of cases. The question is whether 
the deformity directly causes the disease of the nerves, whether both 
arise from a common cause, or whether the two conditions are coinci- 
dental. All authorities agree that there is a closer relation than coin- 
cidence. According to Norris and Oliver, vision is defective in greater 
or less degree from birth. Some are born blind, others have fair vision 
in early life, but slowly sight diminishes until, during adolescence, total 
amaurosis is reached. Some retain a little vision even in advanced 
life. Virchow and Hirshberg attribute the optic neuritis to some intra- 
cranial inflammatory trouble, in most cases probably a pachymeningitis, 







i-k 






V A 




ANOMALIES OF BRAIN AND SKULL 555 

which may also cause the cranial deformity. Friedenwald 4 believes 
the condition is caused by increased intracranial pressure the result 
of premature synostosis of the cranial bones. Opposed to this is the 
fact that in. many cases of microcephalus there is no disease of the optic 
nerve. Necropsies, of which there have been three, throw some light 
on the causation. In Michel's case there was hyperostosis of the skull, 
narrowing of the optic foramina, and thickening of the meninges; in 
Manz's the base of the skull was very irregular and the optic foramina 
were narrow ; in Ponfick's the optic foramina were small. In the first 
two cases the skulls were oxycephalic (steeple-shaped) ; the deformity 
in the third I do not know. In consequence of these post-mortem find- 
ings, Weiss and Brugger examined four steeple-shaped skulls in the 
Heidelberg Institute. They found the optic foramina of normal width, 
but the orbital cavities reduced in size, being very shallow, and the 
alteration in shape being caused by the greater wing of the sphenoid 
forming the posterior instead of the lateral wall of the orbit. The 
dorsum sella? turcica? was enlarged and pushed forward, which they sug- 
gest might have caused pressure upon the chiasm and optic nerves. 
Friedenwald, in discussing their paper, points out that such pressure 
would cause a simple, not a neuritic atrophy. Deformities other than 
oxycephaly may be associated with optic atrophy. In Friedenwald's 
table of nineteen cases, twelve were oxycephalic, three long skulls, one 
leptocephalic, one with broad forehead (synostosis of the sagittal 
suture), one with a bulging temporal region, and one in which the 
deformity is not described. Oliver has published an elaborate paper 
on " Blindness from Congenital Malformation of the Skull." 5 

Oliver also points out that blindness may occur in association with 
deformity of the skull without any disease of the optic nerve. He 
reported the following case. 6 The patient was a four-year-old girl who, 
the mother stated, could see a little till her third year. She showed no 
ophthalmoscopic sign of disease. The eyes were prominent. The head 
was disproportionately small and quite deformed posteriorly. The 
occipital protuberance was almost wanting. The occipitoparietal suture 
was not well closed, the thickened and serrated edges of the occipital 
and parietal bones curving outwardly. Her mental condition was good. 
He believes the blindness was cortical in origin, and suggests as a 
possible cause " stretching and inflammatory processes taking place in 

4 American Journal of the Medical Sciences, May. 1893; Archives of Ophthal- 
mology, 1901. 

5 Proceedings of the American Philosophical Society, vol. xli.. No. 169. 

6 American Journal of the Medical Sciences. January, 1902. 



556 THE EYE AND NEKVOUS SYSTEM 

and around a series of imperfectly made and quite readily disturbed 
tissues in the occipital region." The most frequent cause of the atrophy 
is surely that described by Virchow and Hirshberg. 

PROGRESSIVE FACIAL HEMIATROPHY. 

This affection is very rare. It is most apt to begin before the tenth 
year, is less frequent in adolescence and very rare in old age. In 
92 cases collected by Hermann Steinert, 60 cases occurred in females, 
30 in males, and in two the sex was not mentioned. It has been reported 
twice in parent and child. Its true causes are unknown. The most 
frequent preceding event which could stand in a causal relation is 
trauma of the face. It has followed the acute infectious fevers after 
a longer or shorter period, and has occurred during the course of many 
nervous diseases, — e.g., hysteria, multiple sclerosis, locomotor ataxia, 
epilepsy, and syringomyelia. Its association with none of these diseases, 
however, has been more frequent than can be accounted for by the law 
of coincidence. There seems to be a relationship between it and 
scleroderma. The two diseases occur together with relative frequency, 
Facial neuralgia so often precedes the appearance of the atrophy 
that it may properly be regarded as a symptom rather' than an asso- 
ciated disease. 

Little is known as to its morbid anatomy and the real seat of disease. 
Mendel had one autopsy. The patient had been ill twenty-five years. 
Along with atrophy of the face there was atrophy of the left arm. Post- 
mortem examination revealed proliferating interstitial neuritis of the 
left trifacial, most marked in the second branch. The facial was nor- 
mal, but the left musculospiral was affected in the same way as the 
trifacial. In an atypical case reported by Homen there was a tumor 
pressing upon the Gasserian ganglion and the branches of the trifacial. 
There was not only facial hemiatrophy but also anaesthesia, palsy of the 
oculo-motor nerve, and atrophy of the tongue. There was degeneration 
of all branches of the trifacial nerve, and of the third, fourth, sixth, 
and seventh nerves. Hemiatrophy may also follow injury to the fifth 
nerve. Some authors tend to the view that the seat of the disease is in 
the cervical sympathetic. Thus Seeligmuller reports a case following 
injury in that region. Certain authors regard it as a true skin disease. 
This is surely an error. It is a disease of nutrition and most likely 
depends upon perversion, not mere abolition, of function in the fifth 
nerve or the sympathetic system, or both. Congenital predisposition 
seems to be important for its occurrence, — at least it is most frequent 
in the neuropathic. 



PROGRESSIVE EACIAL HEMIATROPHY 557 

Symptomatology. — The first objective sign of disease appears in the 
skin. A circumscribed spot on the side of the face becomes dead white 
or yellowish and at the same time the skin grows thin. Rarely two 
spots are attacked at once. The disease is most apt to commence in the 
orbital region or the lower jaw. Soon there is a loss of fatty tissue 
under the skin and a shallow pit is formed. The affected area extends 
and involves not only the skin but the subcutaneous tissues and bone, 
and in less degree the muscles. There is never reaction of degeneration 
of the muscles nor complete palsy. The fatty tissue of the orbit disap- 
pears and the eyeball sinks in. The final color of the skin is a shallow 
yellow or brown. The brow and beard fall out or become white. The 
hair of the head is rarely affected. Perspiration may be increased, but 
the secretion of the sebaceous glands lessens or ceases. The disease is 
progressive, but in the greater number of cases ceases at the middle 
line. It may, however, pass over to the other side. The neck muscles 
and the arm of the same side may be involved. Except at the begin- 
ning its progress is very slow, and though recovery never occurs, — i.e., 
the face never regains its natural aspect and form, — the process may 
cease at any time. It may recur. The deformity in an advanced case is 
marked and characteristic. The diseased side is depressed below the 
level of the other from the forehead to the chin. The line of demarca- 
tion is clear-cut. 

There are many minor symptoms. Pain or itching in the affected 
region is frequent. The tongue, hard and soft palate, and the gums may 
atrophy. The teeth may fall out. There is never complete anaesthesia, 
but there may be hyperesthesia. The atrophied skin may feel to the 
patient tense and mask like. Rarely the pupil is dilated or very small. 
There is never disturbance of vision, but taste and hearing may be 
impaired. In very few cases neuroparalytic ophthalmia has been 
observed. Sachs and other authors refer to cases in which there were 
marked spasm of the masseters, clonic, tonic, or both. 

The disease does not shorten life. The only conditions with which 
it can be confounded are congenital facial asymmetry, facial palsy, and 
facial hemihypertrophy of the opposite side. These need only to be 
remembered to avoid error. It may be associated with scleroderma, 
but the differentiation is not difficult. Scattered through the litera- 
ture are reports of atrophy occurring in other parts of the body, similar 
in onset and progress and involving the same tissues. It is probable 
that some of these at least are identical pathologically with this 
disease. 

Treatment has no influence. 



558 THE EYE AND KEKVOUS SYSTEM 



PROGRESSIVE FACIAL HEMIHYPERTROPHY. 

This condition is still rarer than hemiatrophy. It is characterized 
by an overgrowth of one side of the face. The soft parts may be alone 
involved or together with the bones. It usually is congenital, but may 
first appear in childhood or youth. Its causation and morbid anatomy 
are unknown. The earliest case I know of was reported by Stilling in 
1840. The hypertrophy followed an injury which occurred when the 
patient was nine years old. Montgomery's case followed two years after 
an abscess of the cheek. It has followed various illnesses and without 
any apparent cause. The cases reported are too few to draw any con- 
clusions from as to causation. There may be no sign of disease beyond 
the mere increase in size. Only the soft parts with or without the 
mucous membrane of the mouth may be affected, or the bones, or one 
or more of them, may be affected also. Only a part of the face may be 
involved, as the cheek. In Hoffman's case, 7 that of a girl of fourteen 
years, in whom the change was first noticed at two years, there was a 
growth of hair on the upper lip. The skin may be normal or thickened 
and pigmented. Once only has it been recorded as thick and coarse. 
It rarely is hypersemic. There are no characteristic sensory symptoms. 
Berger's case, however, followed an old trifacial neuralgia. Dana's 
case occurred in a young giant. The sebaceous and sweat glands may 
be overactive. There may even be an increase of saliva on the affected 
side. There are no ocular defects nor disturbances of vision. The 
only changes found so far at autopsy have been hyperostosis, uniform 
or in masses, an increase in the adipose tissue, and an atrophy of muscle 
fibres. It is progressive during youth but ceases, or almost ceases, at 
maturity. It never causes death. Once, by pressure of the thickened 
skull upon the brain, it caused epileptiform convulsions. 

OTHER PROGRESSIVE HYPERTROPHIES. 

Occasionally there is seen an hypertrophy of one entire half of the 
body, or of one arm or leg, or finger, or as is reported in one case, one 
side of the face and the corresponding arm and the leg of the opposite 
side. The symptoms are precisely the same as in the facial form and 
it is probable that the pathology is the same. It seems, however, that 
the latter is more frequently acquired than the former. All the tissues 
of the part are -affected, the skin, subcutaneous soft parts, the muscles 
and the bones. Arnheim indeed describes a case in which at autopsy 
the internal organs were found hypertrophied. The right ovary, kid- 

7 Deutsche Zeitschrift fur Nervenheilkunde, vol. xxiv. p. 425. 



ACROMEGALY 559 

ney, lymphatic glands, and even the right side of the heart were 
enlarged. Hymanson 8 found by actual measurement that in his case 
growth was much more rapid on the affected side. We know nothing 
about the cause of the condition, but Anderson gives the following 
views as to its etiology: (1) A congenital lesion of the vasomotor 
centres leading to vascular stasis, (2) a primitive vice of the middle 
lamina of the blastodermic membrane, (3) partial intrauterine 
strangulation of the affected member, (4) an inherent tendency of the 
tissues to appropriate an excess of nutriment. England found at 
autopsy on a child, in whom the left side of the face and head was 
hypertrophied, neurofibromata involving all the cranial nerves. 

ACROMEGALY. 

Acromegaly was first described by Pierre Marie in 1886. Long 
before that time many writers had reported cases of curious deformity 
and strange alteration in physiognomy coming on in youth or adult 
life, and of course giantism was well known ; but Marie was the first 
to separate from these cases of acquired abnormality of anatomic struc- 
ture the type which we now call acromegaly and to show that it is a 
distinct disease with a definite symptomatology and probably due to a 
single cause. It is of interest to oculists because of the ocular symptoms 
due to the associated, if not causative disease of the hypophysis, and to 
neurologists because of the obscurity of its pathology and the occurrence 
of nervous symptoms. 

The most striking feature of the disease, that which makes it pos- 
sible to diagnose a well developed case at sight, is the alteration in 
physiognomy resulting from an overgrowth of the hands and feet, the 
head, jaws, lips, and tongue and a cervico-dorsal spinal curvature. The 
most frequent first subjective symptom is headache, dull or sharp, local 
or general, paroxysmal or lasting. The change in bulk is first called 
to the notice of the patient by the fact that he must wear larger and 
larger shoes or gloves. Even before this, friends may observe an inex- 
plicable alteration in the facial expression. 

Causation. — The predisposing causes are unknown. Direct inheri- 
tance seems to play no part, though a few cases have been reported in 
brothers. It is possible that in them pathological incidents during 
intrauterine life acted as predisposing causes rather than heredity. 
Most frequently the cases are isolated, one in a family. The influence 
of race is unknown, but it seems to be very rare in the American negro, 



Archives of Pediatrics, vol. xx. p. 428. 



560 THE EYE AND NEKVOUS SYSTEM 

Berkley alone, so far as I know, having reported a case. According to 
the tables of Hinsdale, males are more predisposed than females; ac- 
cording to Dana, the two sexes are affected with about equal frequency. 
The period of greatest frequency of onset, so far as that can be deter- 
mined in a disease so insidious in its development, is from the twentieth 
to the thirtieth year. A few cases have been reported in which the onset 
occurred after the seventieth year and recently there have been pub- 
lished instances in which the disease was alleged to be congenital. It 
is by no means certain that these are genuine or that they should not be 
classed elsewhere. They may bear the same relation to the acquired 
disease that infantile cretinism bears to myxoedema. The relation of 
giantism and acromegaly is much discussed. They certainly are not 
the same thing and the latter is not infrequent in the former. 

The exciting causes are also unknown. The rule is for the disease 
to develop without the previous occurrence of anything which could be 
assumed to be a cause. It may follow an acute infectious fever, rheu- 
matism, trauma, childbirth, prolonged emotional strain and, indeed, 
any disease. It sometimes occurs in imbeciles and epileptics. One 
thing is sure, alcohol and syphilis are not exciting causes. If they 
were it would be much more common. I shall discuss the relation 
between disease of the hypophysis and acromegaly under the heading, 
morbid anatomy. 

Symptomatology. — The usual picture is as follows: Some time 
between late adolescence and middle life the hands and feet begin to 
enlarge and the face to alter. At the same time headache begins, is 
much treated and persists. The lips and eyelids become thickened, the 
ears enlarged, the nose broadened, lengthened, and the septum thick- 
ened. The mucous membranes hypertrophy and the tongue becomes 
unwieldy from its increased bulk. While these changes are occurring 
in the superficial tissues, similar ones take place in the bones of the 
skull and especially in the face. The lower jaw enlarges so much as 
to cause prognathism, the line of its circumference extending far beyond 
the upper jaw. The cheek bones become prominent. The voice grows 
hoarse and deep. The skull increases only moderately in circumfer- 
ence, but the fingers lengthen and broaden and become like spades. 
Visual disturbances appear, contraction of the fields or hemianopsia. 
The patient becomes dull, lethargic, stupid, unfit for any work. A 
true melancholia may appear. In the meanwhile the vertebras have 
become affected and cervicodorsal kyphosis sets in. Finally the 
patient dies from the disease of the hypophysis, with symptoms of brain 
tumor or from some intercurrent affection. The duration of the disease 




Fig. 3. — Acromegaly, showing facial deformity and marked dorso-cervical kyphosis. (Case of Dr. 
F. A. Packard. American Journal of the Medical Sciences, Jan.-June, 1892. For photograph of brain 
from this case, see chapter ix, page 428J. 



ACROMEGALY 561 

is very variable, and using this as a means of classification Sternberg 
divides it into three varieties, namely, the benign, which lasts about 
fifty years and in which the changes are very slight, the usual chronic 
form having a duration of from eight to thirty years, and an acute 
malignant form, in which death usually occurs within four years. In 
the last the hypophysis is always sarcomatous. Notwithstanding the 
high mortality of the disease and its tendency to slowly but surely 
progress, in some cases the pathologic process ceases and in that sense 
the patient recovers. The lesions already produced of course remain. 

Even at the expense of a little repetition the symptoms, both 
objective and subjective, deserve individual study. The objective symp- 
toms are the more important in diagnosis. Most striking is the change 
in the general appearance of the patient, caused by the thickening of 
the cutaneous, subcutaneous, and in less degree, the bony structures of 
the hands, feet and skull. As a rule, the enlargement begins simul- 
taneously in all parts, but occasionally one extremity is affected before 
the others. The fingers and toes enlarge more in circumference than 
in length. They become sausage-shaped, and the broadened hands spade- 
like. The disproportion between the much enlarged hands and the 
normal arms and forearms is so marked as to be grotesque. On the 
palms and soles the epidermis and true skin is much thickened and 
pads form. The nails are flattened and striated longitudinally. The 
orbital ridges and the external occipital protuberance are marked. The 
increase in the circumference of the skull is largely due to enlargement 
of the sinuses, but sometimes the cranial bones are thickened. 

The bony changes in the thorax are characteristic. As stated above, 
cervicodorsal kyphosis is common, — sometimes it is so marked that the 
ribs touch the brim of the pelvis. The sternum is thickened and 
widened, the ensif orm being very prominent. The increased thickness of 
the manubrium is important to remember, because the percussion note 
which it* gives may be supposed to be due to a persisting thymus gland 
beneath it. Erb regards sternal dulness as pointing strongly to the 
presence of the thymus gland. This sign is surely not infallible. The 
clavicles and ribs are massive. On account of the changes in the bones 
of the thorax its shape is much altered. Its anteroposterior diameter 
becomes proportionally greater than the lateral. The pelvic bones and 
the great joints of the body are not affected. Except in the so-called 
giant type of the disease the long bones remain normal. The neck is 
thickened and the abdominal walls are sometimes pendulous. 

The ocular symptoms are important. The eyeballs are often prom- 
inent and there may be extreme exophthalmos, due probably to an 
36 



562 THE EYE AKD NEKVOUS SYSTEM 

increase of the fatty tissue behind the bulb. Hinsdale quotes Burchard 
as reporting one case in which the eyeball itself was enlarged. Barely 
severe ocular neuralgia occurs. Nystagmus is sometimes seen. There 
may be partial or complete palsy of the third and sixth nerves. The 
irides may not react to light at all or hemianopic iritic rigidity may 
be present. Some visual disturbance always exists. There may be only 
slight dimness of vision or absolute blindness consequent upon optic 
atrophy due to papillitis caused by enlargement or tumor of the hypoph- 
ysis. The form and color fields may be concentrically contracted or 
there may be true hemianopsia. Disease of the hypophysis and possi- 
bly, as Broca believes, bony changes in the optic foramina consequent 
upon the overgrowth of the lesser wing of the sphenoid bone may pro- 
duce bitemporal hemianopsia, — i.e., blindness in the nasal half of each 
retina. Homonymous hemianopsia has been described by de Schwein- 
itz, among others. The type of hemianopsia present depends upon the 
direction of the pressure upon the optic chiasm and optic nerves. Some- 
times there is total blindness in one eye and hemianopsia in the other. 
The ophthalmoscopic picture is the same as is found in tumor of the 
brain in any region. 

The larynx is enlarged in all directions, the mucous membrane and 
the cartilages thickened, making the voice deep and hoarse. The 
thyroid may be enlarged or diminished, hypertrophied or sclerosed. 
Very rarely Graves's disease occurs. Menstruation in women and 
sexual desire and power in men are apt to disappear early. At first 
muscular power is retained or even increased, but later there is marked 
weakness. The knee-jerk is usually normal. It may on account of 
complications be absent, increased, or unequal on the two sides. Elec- 
tric conductivity is increased. The urine frequently contains albumin 
and glucose and may be enormously increased in quantity. The blood 
presents no noteworthy changes. Disturbances of sensibility do not 
occur in uncomplicated cases, but hysterical hemiansesthesia has been 
noted. Extreme hunger and thirst are not uncommon. . There is often 
cardiac enlargement with dilatation causing paroxysmal palpitation. 
The characteristic mental state is one of apathy, lack of energy, and 
marked sleepiness. There is almost always some emotional depression 
which may pass into a true melancholia. Some patients become simply 
a little silly, mildly demented, and unduly cheerful and good natured. 

Morbid Anatomy and Pathology. — The skin, subcutaneous tissues, and 
the bones in the affected regions of course always show marked signs 
of disease. There is a true hypertrophy of the skin and the sweat and 
sebaceous glands, of the arterial walls, and of the sheaths of the 




Fig. 5.— Showing deformity of hand. (Case of Dr. F. A. Packard.) 





Fig. 6.— a. Photograph of the patient at twenty-two years of age. b. Photograph at forty-five years of 
age. (Case of Dr. F. A. Packard.) 




Fig. 7.— Portion of skiagraph of left hand of Dr. Walton's case of acromegaly. (Actual size.) 



ACKOMEGALY 563 

cutaneous nerves. In the bones the periosteum is thickened, the grooves 
of the blood-vessels deepened and enlarged, and in the skull and spine 
small extoses are present. The bones are thickened, and since this does 
not involve the whole bone symmetrically, deformity ensues. The lower 
jaw is always most affected. In the spine the vertebrae are ankylosed 
by bony overgrowth. The smaller joints present appearances similar to 
those seen in arthritis deformans. In a case of Broadbent's, X-ray exam- 
ination showed that there was enlargement of the soft parts only, the 
bones being of normal size. This may mean, however, not that disease 
of the bone is not an inherent part of acromegaly, but that in this case 
the skin became affected first, as indeed may be the rule. 

The organ most discussed in all studies on acromegaly is the hypoph- 
ysis. It is more frequently found diseased than any other organ in the 
body, and not a few authorities hold that if it be normal acromegaly 
cannot occur. They explain the few cases in which normal hypophyses 
have been found at necropsy by claiming errors in clinical diagnosis. 
The following seem to be the facts in the matter. The hypophysis is 
diseased in the great majority of cases if not in all. It may be hyper- 
trophied or the seat of a tumor. On the other hand, disease of it com- 
pletely destroying its glandular structure and, of course, also its rudi- 
mentary nervous structure, may occur without any symptom of acro- 
megaly appearing. Such disease may occur at any period of life and last 
for quite a long time. For example, a patient of mine who had adiposis 
dolorosa showed at necropsy a tumor as large as a walnut. Her illness 
had lasted many months. The kind of disease affecting the hypophysis 
seems to be a matter of no moment since hypertrophy and tumors of 
many kinds occur. Some writers hold that simple hypertrophy of the 
hypophysis is only a symptom of the disease, and not causative. Exper- 
imental destruction of it in lower animals does not produce acromegaly. 
This may, however, be due to the fact that death is apt to follow very 
quickly, that the hypophysis may have other functions in lower ani- 
mals than in man, and that destruction of function need not, indeed 
ought not, to cause the same symptoms as perversion of function, such 
as may occur in a diseased but not totally destroyed organ. Altogether 
it is best to confess ignorance and admit that the true relation between 
acromegaly and hypophyseal disease remains unexplained. Others of 
the so-called ductless glands are often found diseased, notably the thy- 
roid. The thymus, which should not be discoverable in an adult, is 
often present and the seat of a tumor. The ovaries and testicles are 
often atrophied. Slight and probably coincidental changes in the spinal 
cord, degeneration of the posterior and lateral tracts have been found. 



564 THE EYE AKD NEKVOITS SYSTEM 

The spinal and sympathetic ganglia have, when examined, shown hyper- 
trophy. Syringomyelia has been reported, but surely cannot have any 
causative influence. A few investigators have endeavored to throw light 
on the pathology of acromegaly by chemical studies? but so far with 
little success. Edsall and Miller, for example, made a very careful 
study of the urine in two cases, but could only show that there is " a 
growth of abnormal bone rather than a mere abnormal growth of bone, 
and that there are very marked abnormalities" [chemical] " in the soft 
tissues." All explanations of acromegaly heretofore offered have been 
purely hypothetical, except that of its hypophyseal origin which, while 
not proven, certainly has evidence in its favor. 

Differential Diagnosis. — A fully developed typical case of acromegaly 
can be diagnosed at sight. There are, however, several diseases which 
may in fact be pathologically nearly related to it and which may be 
mistaken for it unless a careful examination be made. Myxoedema, for 
example, has a slight superficial resemblance to it, but the myxoedema- 
'tous swelling involving the entire body, the local tumefactions partic- 
ularly in the suprascapular region, the puffed face with obliteration of 
the lines of expression, and the brittle, thin hair are points quite suffi- 
cient to serve for diagnosis. Many giants are acromegalic, but in simple 
giantism there is symmetrical overgrowth of the entire body and not 
of the extremities alone. So-called partial giantism, hypertrophy of 
individual extremities, is recognized by its limitation to one part. In 
osteitis deformans (Paget's disease) the shafts of the long bones are 
chiefly affected, and in the head the bones of the cranium, not those of 
the face. In Paget's disease* the face is triangular with the base up, in 
acromegaly it is egg-shaped. In Paget's disease also changes in the soft 
tissues are not present and the symptoms rarely appear before the 
fiftieth year. In leontiasis ossea the cranial bones alone are affected. 
Adiposis dolorosa is distinguished by the local swellings, the pain, the 
absence of deformity, and of ocular symptoms. The most difficult 
differential diagnosis of all is that from pulmonary hypertrophic osteo- 
arthropathy. This disease also was discovered by Marie. In it the 
bones are more affected than the soft parts. The wrists are greatly 
deformed while the lower jaw escapes. Kyphosis is rare, scoliosis fre- 
quent. The hands are paw-shaped. There is always some pulmonary 
affection. X-ray examination is useful in diagnosis. 

Treatment. — Nothing has been found which influences the course of 
the disease. Thyroid and hypophyseal extracts have no effect. At- 
tempts to remove the tumor of the hypophysis have been followed by 
death in a short time. 




180 360 



210 




27 



Observed in 1885. 



f ,d totf>e-JV.B'p 





Observed in 1S92. 
Fig. 8.— Visual fields. (Dr. F. A. Packard's case of acromegaly.) 



CHAPTEE XV. 

DISEASES OF THE SPINAL COED AXD SPIXAL XEEYES. 

By T. H. WEISENBUBG, M. D. 

MYELITIS. 

Hyelitis is inflammation of the substance of the spinal cord. We 
have the acute, subacute, and chronic forms, depending upon the mode 
of the onset. W lien the inflammation involves the whole thickness of 
the cord for one or more segments, it is called transverse myelitis ; if 
more extensive in continuous areas, vre have the diffuse type ; and if the 
process is at various levels, it is called a disseminated myelitis. 

Etiology. — The exact cause of acute myelitis is not known, but it is 
believed to be an acute infectious disease due to a specific germ. In 
several instances staphylococci and streptococci have been found in 
the cerebrospinal fluid, and the pneumococcus has been found in a 
case of disseminated myelitis. Exposure to cold and wet, overexer- 
tion, and the acute infectious diseases, such as smallpox, diphtheria, 
typhoid fever, pneumonia, and gonorrhoea, some of the chronic blood 
conditions, and even gout, may cause myelitis, while syphilis is one of 
the most frequent causes. 

Pathology. — Microscopically, the appearance depends upon the 
stage of the disease. In the acute period the blood-vessels are dilated 
and filled with red and white corpuscles. Around the vessels are found 
leucocytes and accumulations of round nuclei, and occasionally hemor- 
rhages are seen. The nerve-cells are in various stages of disintegration. 
The axis cylinders are swollen and their myelin sheaths destroyed. The 
neuroglial' fibrils are swollen, and their nuclei are much increased in 
number. Granular cells are found in abundance in the neuroglial' tissue 
and around the blood-vessels. 

Symptoms. — The symptoms of acute or subacute myelitis, with 
which we are concerned, depend upon the part of the spinal cord affected. 
Usually the dorsal cord is most intensely involved. The symptoms 
may come on rapidly, sometimes in a few hours, or may come on grad- 
ually, lasting for some weeks or months. There may be a feeling of 
malaise, and even a rise of temperature, and the patient may complain 
of a feeling; of numbness or of a tingling; sensation in the limbs, some- 
times, also, of a pain in the back. Following this, or perhaps without 

565 



566 THE EYE AND NERVOUS SYSTEM 

these pains, there is a feeling of weakness in the extremities, which is 
rapidly followed by complete flaccid paralysis. At times a total paraly- 
sis comes on within a few hours. Sensation in all forms is much 
affected, the upper limit of the anaesthesia depending upon the location 
of the lesion. The reflexes may be lost at first ; but in a short time the 
limbs will become spastic, the reflexes, such as the patellar and the 
Achilles jerks will be much increased, and the Babinski phenomenon is 
obtained. The vesical and rectal sphincters are involved from the 
beginning, there being at first retention of urine and constipation, fol- 
lowed by incontinence. Trophic disturbances, such as bed-sores, soon 
appear. Wasting of muscles and the reactions of degeneration are 
obtained, provided the cells of the anterior horns supplying these parts 
are destroyed. Lumbar myelitis causes somewhat different symptoms. 

If the infection is intense, the disease extends rapidly, soon affect- 
ing the cells supplying the respiratory muscles and causing death. If 
the patient survives, the course of the symptoms will depend upon the 
severity of the lesion. Usually sensation will return before motion, and 
the patient will gradually show the symptoms of spastic paraplegia. 

Ocular Symptoms. — Inflammation of the optic nerve accompanying 
myelitis is rare. Steffan and Erb, 1 in 1876, first described such an 
occurrence. Katz, 2 in 1896, collected twenty-one cases. Since that 
time seventeen have been recorded, to which the author is able to add 
one case of his own. This makes a total of thirty-nine cases ; and when 
the large number of instances of myelitis are considered, the rarity of 
the affection is shown. (See Chapter VIII, p. 375). 

In the majority of instances the disease first manifests itself in a 
rapid diminution of vision, mostly in both eyes, but sometimes only 
in one. Often some time elapses before the second eye is affected, 
which is frequently not until the first is on the way to recovery. 

The diminution of vision is usually rapid. In one case, after 
twenty-four hours there was complete amaurosis. In other instances 
a few days, and sometimes a number of months, elapse before there 
is complete loss of vision. In the author's case one month passed before 
there was complete blindness. 

It is not always necessary to have subjective symptoms of diminu- 
tion of vision, for in a number of reported cases the patients did not 
complain of such phenomena, and even the ophthalmoscopic examina- 
tion may be negative, the diagnosis of optic neuritis being made by the 
microscope. 

x Arch. f. Psych, u. Nervenkr., vol. x. No. 1. 
1 Arch. f. Ophthalm., vol. 42. 



MYELITIS 



567 



Usually the symptoms are ushered in by pains in the orbit and in 
the frontal region of the affected side. They are increased by movement 
of the eyeball, and there is sometimes acute pain on pressure. These 
pains have been variously explained. Some authors believe that they 
are due to an inflammation of the optic nerve sheaths or to a periostitis 
of the optic foramen. They have also been attributed to that form of 
inflammation of the optic nerve known as retrobulbar neuritis. Most 
probably they are due to the irritation of the sensory branch of the fifth 
nerve, which supplies the optic sheath. (See Chapter XVIII, p. 743.) 

In the majority of instances the optic neuritis precedes the mye- 
litis, sometimes by a few days, or from three to five months. The 




Fig. 1.— Case of meningo-myelitis with bilateral optic atrophy secondary to optic neuritis. 
Position of hands shows extent of power of elevation. 



symptoms may come on together, or the optic neuritis may follow the 
myelitis, as in the author's case, after three months. 

The condition of the pupils varies. Usually they are wider than 
normal and the reaction to light is lost. This has been explained by 
the existing amaurosis. The pupils, however, may be unequal, or one 
or both may be myotic. If the myelitis involves the cervical cord, espe- 
cially the eighth cervical and first dorsal segments, there may be a con- 
traction of the pupil, narrowing of the palpebral fissure, and enoph- 



568 THE EYE AND NEKVOUS SYSTEM 

thalmus, due to paralysis of the cervical sympathetic. Irritation of 
the sympathetic produces widening of the palpebral fissure, exophthal- 
mos, and dilation of the pupil — as in one case (Bielchowsky), in which 
exophthalmos and swelling of the lids were seen. Nystagmus has been 
mentioned but once, by Brissaud and Brecy ; 3 it being vertical in 
direction. Except in one instance, in which there was redness, nothing 
pathological has been found in the external part of the eye. 

Disturbances of the fields of vision and of color-sense are not 
numerous, because the blindness is usually rapid and in all parts of the 
field. Central scotoma, a concentrically contracted field of one eye, 
or a quantitative light disturbance in a part of the field, have been 
described. Disturbances of color-perception have been mentioned a 
number of times, as well in the beginning as in the end of the disease. 
In one instance a unilateral color-blindness was found after the optic 
neuritis had disappeared. 

Involvement of the other cranial nerves is rare. Mention is made 
of it in only four cases. The facial nerve was involved in one instance, 
while in another both internal recti muscles and the seventh and twelfth 
nerves were implicated. In the other two cases there was paralysis of 
both internal and external recti muscles, ptosis, and paresis of the facial 
and hypoglossal nerves. In several reported cases some of the peripheral 
nerves were affected, such as the median, sciatic, and external popliteal ; 
microscopical examination showed an acute neuritis, similar to the 
inflammation of the cranial nerves. 

Ophthalmoscopically, we usually find in the beginning of the neu- 
ritis more or less swelling of the papillae, tortuosity of the vessels, and 
blurring of the edges of the disk. This is by no means always the case, 
for the ophthalmoscopical findings may be negative all through the dis- 
ease. The retina is usually not affected. Haziness in the region of the 
macula and an increasing pigmentation of the pigment epithelium have 
been described. Microscopical examination does not explain the latter 
condition. Some authors consider these changes as due to a degenera- 
tion of the retina. 

Both optic nerves are involved in the majority of instances. Two 
cases, however, are recorded in which only one of the nerves was dis- 
eased, but in one of these cases there was an overfilling of the vessels 
of the opposite eye. 

After the disturbance of vision has reached its highest stage — 
this lasting, on an average, about two weeks — there is usually an im- 

3 Rev. Neurol., Jan. 30, 1904. 



MYELITIS 569 

provement if the case is not fatal ; and, rarely, a return of vision. Only 
three cases are on record in which the return of vision was complete. 
Usually complete blindness in both eyes results. 

Ophthalmologically, in about half the cases there is a resulting 
white atrophy of the optic nerves ; neuroretinal changes have been ob- 
served in two instances, and in three the ophthalmological findings after 
the subsidence of the ocular symptoms were completely normal, but in 
none of these three cases were the symptoms severe. 

Remissions of the symptoms have been noted in five instances. 
These occurred simultaneously in the eye and in the cord, and have 
been explained in various ways. Elschnig 4 believes that the patho- 
logical process in the optic nerve is in the nature of a primary interstitial 
neuritis ; and that the inflamed interstitial tissue exerts a mechanical 
pressure upon the nerve-fibres and the vessels, thereby interfering with 
their function. Should the inflammation subside, function would be 
restored. Bielchowsky, however, thinks that the process is a primary 
parenchymatous neuritis; and that the remissions occur because the 
toxin causing the disease is diminished, and the axis cylinders regener- 
ate, thus restoring function. 

Of the 39 reported cases of myelitis associated with optic nerve 
changes, 20 have been with autopsy. It is striking that in nearly all 
the myelitic process involved almost the whole extent of the spinal 
cord. The process in all the cases was either acute or subacute, both 
in the spinal cord and in the optic nerves, and not chronic. 

In most instances the nature of the inflammation is that of an acute 
disseminated myelitis. Transverse myelitis is of rare occurrence. His- 
tologically, the myelitis is either in the nature of an acute diffuse paren- 
chymatous inflammation or a combined parenchymatous and inter- 
stitial process. Bielchowsky, 5 in a thorough microscopical examination 
of four cases, found three distinct pathological processes. In his first 
case the inflammation was of the acute hemorrhagic type, affecting the 
whole extent of the spinal cord. Here the inflammation was as much 
interstitial as parenchymatous. In the second case, the myelitis was 
diffuse and of a primary parenchymatous nature, and affected prin- 
cipally the white matter. The nerve-fibres were everywhere destroyed, 
the myelin being partially or completely broken up, and the axis- 
cylinders were degenerated. The lumina of the blood-vessels were not 
overfilled, but the walls, especially the adventitia, were full of granu- 



Arch. f. Augenheilkunde, vol. xxvi. 

Myelitis u. Selmervenentziindung, Berlin. 1901; V. v. S. Karger. 



570 THE EYE AND NERVOUS SYSTEM 

lar cells. The glia fibres seemed normal or perhaps a little swollen, and 
the neuroglia cells were increased in number. In the meshes of the glia 
tissue, however, there was an enormous number of granular or fat- 
cells, these being also very numerous around the blood-vessels. A slight 
proliferation of the endothelial cells of the intima was likewise present. 
In the third and fourth cases the inflammation seemed to be limited 
largely to the posterior and lateral tracts. Histologically, the process 
was similar to that in the second case. In the majority of instances of 
myelitis with optic neuritis, the myelitis is such as is reported in Biel- 
<?howsky's second case — notably so in the cases recorded within recent 
years, in which microscopical examinations have been made by modern 
methods. Some authors, however, as Elschnig, interpret the inflamma- 
tion as being primarily interstitial in nature. 

The histological process in the optic nerve is always similar to that 
in the spinal cord. There is either an acute hemorrhagic inflammation 
or a primary diffuse parenchymatous neuritis. In the latter case, how- 
ever, the inflammation does not present the diffuse areas seen in the 
spinal cord, but a continuous lesion. This is due to the merging of the 
adjacent softened aress. 

In most instances the optic nerve is involved throughout its whole 
extent, from the papillae to the chiasm and through the whole thick- 
ness of the nerve. This is not always so, however, for there may be 
in the periphery of the nerve a small band of normal fibres. The 
chiasm is involved in most cases, and the process may extend back of 
the chiasm into the optic tracts. Secondary degeneration may be traced 
partially into the optic tracts, but the primary optic centres — viz., the 
external geniculate body, the pulvinar of the optic thalamus, and the 
anterior corpora quadrigemina — have never been found diseased. The 
fibres of Gratiolet and the cuneus have also never been found in a dis- 
eased condition. 

A cross section of the diseased optic nerve will always show a widen- 
ing of the interfascicular septa; an increase in the number of neu- 
roglia cells ; a large number of flattened granular cells in the meshes of 
the glia tissue and around and in the blood-vessels ; and a destruction 
of the nerve-fibres. As a rule, the pial covering of the optic nerve is 
not altered. It has been observed to be diseased, however, in two in- 
stances ; and here it was probably local in origin, for the accumulation 
of nuclei was opposite the point of greatest inflammation of the optic 
nerve. The endothelial cells covering the subarachnoid spaces were also 
increased in these two cases. 

If the whole optic nerve is equally diseased, it is impossible to 



MYELITIS 571 

determine whether the process is of retrobulbar origin or not. In the 
majority of instances, however, the optic neuritis is retrobulbar in 
origin, for in an examination of the limits of the pathological process 
— as in the chiasm — recent changes may be found. The inflammation, 
however, need not implicate both the intraorbital and intracranial 
portions of the optic nerve ; for in a number of cases the intraorbital 
part of the nerve was perfectly normal, — the inflammation beginning in 
the central part of the nerve, before its decussation, and involving the 
chiasm and the optic tracts to a limited extent. 

Marked neuritic atrophy of the papilla and of the surrounding part 
of the retina has been described by Dalen. 6 

Examination of the other involved cranial nerves showed an intense 
acute neuritis. The inflammation was not secondary to a nuclear change 
nor to an inflammation of the pia, and there was no secondary degener- 
ation. 

In two instances — those of Elschnig and of Weil and Gallawardin 7 
— there was a slight diffuse interstitial encephalitis. In no case was 
there basal meningitis. In the medulla oblongata, except for the usual 
ascending secondary degenerations, nothing abnormal was found. 

Relation between the Myelitis and the Optic Neuritis. — Several 
theories have been advanced to explain the occurrence of optic neuritis 
with myelitis. Wharton Jones believed that the optic neuritis was 
caused by a circulatory disturbance, due to involvement of the cervical 
sympathetic. Allbutt is of the opinion that the optic neuritis is due 
to an ascending basal meningitis ; while still other authors believe that 
an accompanying intracranial lesion causes the optic neuritis. 

From the evidence of recent pathological findings, all these theories 
may be dismissed. In most instances the optic neuritis precedes the 
myelitis ; so the hypothesis of an optic neuritis secondary to a cervical- 
sympathetic lesion is not tenable. Again, there is not the accompanying 
sympathetic phenomena; nor does a sympathetic lesion produce optic 
neuritis. 

Allbutt' s theory may be readily dismissed, for no basal meningitis 
has been found in any recorded case. So far no secondary intracranial 
lesion has been observed. In only two instances was there cerebral 
involvement, and in these it was in the nature of a diffuse encephalitis. 

Taylor and Collier 8 have recently attempted to show that the optic 
neuritis is always due in these cases to a lesion of the cervical part of 

6 Arch. f. Ophthalm., vol. 48. 

7 Rev. Neurol., Oct. 31, 1903. 
"Brain, 1901. 



572 THE EYE AND KERVOUS SYSTEM 

the spinal cord ; and that in every one of the reported cases the patho- 
logical process involved the cervical cord. A careful analysis, however, 
does not seem to bear out this statement. In sixteen of the twenty 
autopsies the lesion did extend to the cervical cord ; but in the majority 
of these the symptoms seem to have indicated that the pathological 
process had begnn in the dorsal cord and extended upwards. Clinically 
there was no analogy between the appearance of the optic neuritis and 
the myelitis. In fact, as before stated, the optic neuritis generally pre- 
cedes the myelitis. In support of this theory it may be said that in 
several reported cases the optic neuritis was most marked on the side 
corresponding with the greater involvement of the spinal cord, the 
lesion in these cases being principally in the cervical cord. Taylor and 
Collier also believed that the inflammation must be a partial one ; that 
is, not involving the whole thickness of the cord. 

In view of the fact that the histological process is alike in the spinal 
cord and in the optic nerve, we must come to the conclusion that the 
processes are identical and due to the same cause — probably a toxin 
that exerts its influence equally on the spinal cord and on the optic nerve. 
Why this toxin should pick out the optic nerve is, however, a question. 
It may be explained on the theory of locus minoris resistentice. The 
optic nerve has the same embryological origin as has the spinal cord, 
and it may be that the toxin that acts upon the spinal cord has a 
selective action upon the optic nerve. 

^Prognosis. — As a rule, the prognosis in these cases depends upon the 
progress of the myelitis, for improvement in the spinal-cord symptoms 
always indicates an improvement in those of the optic nerve. 

Treatment. — Treatment is usually unavailing. When a history of 
syphilis is obtained, which is in about one-third of the cases, a combined 
antisyphilitic treatment is indicated; but, as a rule, this is of no 
benefit. The bed-sores should be carefully looked after, and the con- 
tracted limbs kept in good condition. Catheterization is commonly 
indicated. 

SYRINGOMYELIA. 

As a rule, the symptom complex of syringomyelia is so typical that 
the diagnosis does not offer much difficulty. The disease generally 
begins early in life and is chronic and progressive. The symptoms may 
be divided into three forms : first, the atrophy of the muscles of the 
upper limbs ; second, the disturbances of sensation, and, third, vaso- 
motor and trophic phenomena. 

The atrophy usually begins in the small muscles of the hand and 
may involve all the muscles of one or both upper limbs, but is not 



SYEIXGOMYELIA 573 

uniform. Fibrillary twitching may be present and electrical reactions 
of degeneration may be obtained. 

Sensation for touch is generally preserved, but pain and tempera- 
ture sensations are lost. This dissociation of sensation may be present 
in one or both upper limbs and part of the trunk, depending upon the 
part of the spinal cord involved, but it is usually more marked on one 
side. 

Vasomotor and trophic phenomena, as cyanosis of the hand, oedema, 
arthropathies, and deformities of bone, are common. 

The lower limbs may be spastic, due to secondary degeneration of 
the pyramidal fibres, or may be paralyzed, due to direct involvement of 
the lumbar cord, but this is exceptional. If the disease involves the 
medulla oblongata and pons, we may have symptoms of cranial nerve 
palsy, as atrophy of the tongue, disturbances of respiration, laryngeal 
palsy, deafness, facial paralysis, and symptoms of involvement of the 
different ocular nerves. 

Pathology. — The cause of this symptom complex is a cavity or a 
gliomatous tumor formation in the substance of the spinal cord, which 
generally involves its centre, the posterior columns, or the posterior 
horns. It hardly ever involves the anterior part of the cord, and may 
be unilateral throughout its whole extent. The cavity is usually largest 
at the cervical cord and ends in the lower thoracic region, thus explain- 
ing the preponderance of symptoms in the upper limbs. It has been 
thought that the cavity does not extend higher than the nucleus of the 
seventh nerve in the pons, but Spiller has observed an instance in 
which the cavity extended as high as one cerebral peduncle. It is 
important to remember this fact, especially when we consider the 
cause of the different ocular nerve palsies. The cavity is filled during 
life with cerebrospinal fluid. A congenital defect in the formation of 
the spinal cord is probably the basis of the disease. 

Ocular Symptoms. — Eye symptoms are uncommon. We shall con- 
sider in order involvement of the optic nerves, the ocular muscles, and 
the pupils. Optic neuritis has been recorded by Saxer, 9 Billiard and 
Thomas, 10 and by Thorington and myself. 11 In all of these cases a 
complicating internal hydrocephalus was probably at fault, as was 
shown by the necropsy findings in the first two. It is well to remember 
that internal hydrocephalus is not an infrequent complication of 
syringomyelia, and that many of the cranial-nerve palsies which may 

9 Beitrag. z. path. Anat. u. z. allgem. Path., vol. xx. No. 11. 
"American Journal of Medical Science, 1899. 
"Ibid., Nov. 1905. 



574 THE EYE AND NERVOUS SYSTEM 

be present in syringomyelia are due to the pressure of the cerebrospinal 
fluid. This was the case in the above-mentioned instances, in which 
both external recti and the facial nerve were diseased. Hoffmann sug- 
gests that a tumor situated in the medulla oblongata, which may be 
the starting point of a syringomyelic cavity, may cause optic neuritis. 
This undoubtedly may occur and it is probable that in some of the 
cases of secondary optic atrophy this may be the explanation. 

Primary optic atrophy occurs less often in syringomyelia than in 
any other spinal-cord disease. Maixner (quoted by Schlesinger), in 
thirty-two cases, found optic atrophy in three. In one case under my 







. 


i^ijfl 


1 - 




w* * * • 


B^^^^^k » ill J 


if* • * *■ m 









, t 



Fig. 2.— Case of syringomyelia with optic neuritis and bilateral external rectus paralysis ; also 
showing position of head. (Reported with Dr. James Thorington.) 



observation gray degeneration of the optic disk occurred in one eye, the 
other fundus being fairly normal. This is unusual, for, when disturb- 
ances do occur, they usually involve both optic nerves equally. It mus,t 
be remembered, however, that tabes dorsalis rarely complicates syringo- 
myelia, and when primary optic atrophy is present this disease should 
be excluded. 

The ophthalmoscopic findings are usually negative. The disks may 
be pale, but always in their whole extent, differing from the temporal 
paling so often seen in multiple sclerosis. The fundi in two of my 
cases were gray in color. Vision, as a rule, is not interfered with, but 
if disturbances occur, both eyes are equally affected. Neither scotoma 



SYKI2s T G0MYELIA 



575 



nor disturbances of light and color sense have been recorded in this 
disease. 

A concentric restriction of the visual fields, especially for colors, and 
particularly for green, sometimes occurs. Schlesinger 12 collected from 
the literature one hundred and thirty cases, in which this symptom was 
present in thirty-eight. The examinations were perimetric. Of these, 
twenty-four had no symptoms of hysteria. The contraction of the visual 
fields was not severe, and consisted in a peripheral defect, especially for 
green. He concludes that in most cases of syringomyelia the visual 
fields are normal, that the contraction of the fields may be due to a 
concomitant hysteria, and that in a small number of cases of syringo- 




Fig. 3.— Case of syringomyelia with optic neuritis, showing lateral curvature. 
(Reported with Dr. James Thorington.) 



myelia not complicated with hysteria, alterations in the visual fields 
may occur. 

Other authors, however, do not believe that in an uncomplicated case 
with negative ophthalmoscopic findings visual contractions can occur, 
and ascribe these symptoms to hysteria. In five cases under my obser- 
vation, in which a perimetric examination was made, no contraction of 
the visual fields was noticed. Schlesinger explains the contraction by 
an intracranial complication, probably a hydrocephalus. If this is so, 
we might expect that these symptoms would vary on different exami- 

12 Die Syringomyelic, Leipzig u. Wien, 1902. 



576 THE EYE AED NERVOUS SYSTEM 

nations, but this is not mentioned. In the eight cases recorded by Dejer- 
ine and Tuilant, 13 who were the first to call attention to the restriction 
of the visual fields, an examination a year later still denoted the same 
condition. 

Paralysis of Ocular Muscles. — Schlesinger, in a study of three hun- 
dred cases from the literature, found paralysis of the ocular muscles 
in thirty-three, approximately in 11 per cent, of the cases. It occurs 
less often than in multiple sclerosis and tabes dorsalis. The paralysis 
generally comes on early in the disease and may be transient, but later 




Fig. 4.— Case of syringomyelia with partial ptosis of right upper lid. 

in the disease may become permanent. These transient paralyses 
resemble those of syphilis and tabes. 

The abducens nerve is more often involved than the other cranial 
nerves, and more often the involvement is unilateral. The oculomotor 
nerve is usually only partially involved, as shown by a ptosis. This 
is present in the patient whose photograph is given. The ptosis may 
be slight and may vary from day to day, as in the patient in the photo- 
graph, in whom the ptosis varied considerably on successive examina- 
tions. This variability in the amount of paralysis, as shown by the 
abducens nerve, seems to be characteristic of syringomyelia. 

The ptosis which is due to the third nerve involvement must be 
differentiated from the ptosis due to sympathetic paralysis. It is well 

13 Semaine Med., 1891, No. 6. 



SYRINGOMYELIA 577 

known that in sympathetic paralysis all of the symptoms need not be 
present, and we may have in both conditions only a ptosis. An appli- 
cation of cocaine will, in sympathetic paralysis, not dilate the pupil. 

If the syringomyelic cavity extends high up into the pons, we may 
have complete chronic external ophthalmoplegia. Retraction of the ocu- 
lar muscles appears late in the disease. 

A direct involvement of the ocular nuclei or of their intramedullary 
fibres by the cavity will explain the ocular palsies, providing there are 
in these cases some other symptoms of bulbar paralysis. If they are not 
present, an internal hydrocephalus is probably the cause. The tran- 
sient ocular palsies and variability of the ptosis may be explained by 
the difference in pressure of the fluid in the syringomyelic cavity. 

According to Schlesinger, nystagmus and nystagmoid movements 
of the eyeball in syringomyelia occur in about 15 per cent, of the cases. 
Of three hundred and thirty cases, fourteen had true nystagmus and 
thirty-seven nystagmoid movements. Schlesinger states that the nys- 
tagmus comes on early in the disease, but it may be a late manifestation. 
The movements may be horizontal, vertical, diagonal, or rotatory. Dis- 
turbance of vision in nystagmus never occurs, but in a number of in- 
stances the fixing object was said to be fluctuating. 

The nystagmoid movements are far more common than those of true 
nystagmus. They are obtained only on lateral deviation. 

The cause of the nystagmus and the nystagmoid movements in this 
disease are not known, but various theories are given. Some authors 
believe that they are an accidental complication of the disease, but it 
may be argued against this hypothesis that the nystagmus comes on as 
the disease appears. Schlesinger believes that it is due to the pressure 
of hydrocephalus, while other authors believe that it is congenital. 

In the different diseases in which nystagmus is a symptom, such as 
Friedreich's disease and in syringomyelia, a congenital anomaly in the 
formation of the brain and spinal cord is thought to be the cause. 
Multiple sclerosis also is believed by some to be produced in the same 
way. In these diseases the symptoms do not appear until some con- 
tributory cause starts the pathological process. Nystagmus appears at 
the same time. It is a striking fact that in syringomyelia, in the cases 
in which nystagmus occurs, there are no disturbances of vision, accom- 
modation, or in the pupillary reflex, this arguing against an acquired 
lesion. It is probable, therefore, that the cause of the nystagmus in 
these diseases is some congenital maldevelopment, and that this symp- 
tom does not appear until the symptoms of involvement of the rest of 
the brain and spinal cord present themselves. 
37 



578 THE EYE AND NEKVOUS SYSTEM 

Pupillary Symptoms. — The pupils in syringomyelia are usually not 
affected, but myosis and differences in the size of the pupils sometimes 
occur. In two hundred cases studied by Schlesinger pupillary differ- 
ences were found in twenty-four. The pupillary light reflex is not 
often absent, and yet a number of such cases have been recorded. Dejer- 
ine and Mirallie record an instance where the light reflex was absent on 
one side only. It may be assumed that when the pupillary light reflex 
is disturbed or absent, we may have an accompanying tabes or general 
paresis. 

Paralysis of the sympathetic may be caused by a cavity in the lower 
portion of the cervical cord. Sympathetic symptoms are generally uni- 
lateral, but may be bilateral. Such symptoms are narrowing of the 
palpebral fissure, myosis, enophthalmos, and disturbances of the secre- 
tions. As a rule, we do not have all of these symptoms in sympathetic 
paralysis, and we may have only myosis with ptosis without involvement 
of the secretory functions. These symptoms are caused by the in- 
volvement of the sympathetic fibres which ascend in the spinal cord 
from the ciliospinal centre in the first dorsal segment to the medulla 
oblongata. 

Involvement of the Fifth and Seventh Nerves. — The descending 
sensory portion of the trigeminus nerve is sometimes involved in 
syringomyelia. This is first manifested by paresthetic phenomena, later 
by pains, and finally by anaesthesia. A disturbance in the secretion of 
tears and of saliva has been noted in a few instances. The secretion of 
tears is sometimes increased and appears on laughing or on irritation 
of the nasal mucous membranes on the side opposite the anaesthesia. 
The corneal and lachrymal reflexes are lost. Trophic disturbances are 
uncommon. This may be due to an incomplete paralysis of the trigemi- 
nus, but even in total paralysis trophic disturbances may not occur. 
This is probably due to the nature of the lesion, for it has been shown 
that an irritative condition, either in the pons or in the Gasserian 
ganglion, may produce a keratitis. According to Gowers, 14 a greater 
irritation is necessary to produce a corneal ulcer if the lesion involves 
the fifth-nerve fibres within the pons or the sensory root than if the 
lesion is at or in front of the Gasserian ganglion. 

The facial nerve is sometimes involved in syringomyelia. The 
paralysis may be transitory. All or only part of the nerve may be 
diseased. If the upper portion is much involved, it causes a lagoph- 
thalmos. 

14 Diseases of the Nervous System, vol. ii. 217. 



SPINAL CORD DISEASES 579 



THE RELATION OF OCULAR SYMPTOMS TO 
SPINAL-CORD DISEASES. 

In the following pages will be considered the various ocular dis- 
turbances occurring in conjunction with certain spinal-cord diseases, 
such as primary lateral sclerosis, amyotrophic lateral sclerosis, postero- 
lateral sclerosis, and the subacute combined system degenerations which 
sometimes complicate pernicious anaemia. Primary ascending unilateral 
paralysis will also be considered in this connection. As a rule, ocular 
changes are rare, but optic-nerve atrophy has been recorded in all of these 
diseases, as well as pupillary phenomena. 

To understand the relation of the optic-nerve atrophy to the various 
spinal-cord diseases, we must first clearly understand the nature of 
these spinal cord affections. The belief is gradually gaining ground that 
such diseases as primary lateral sclerosis and amyotrophic lateral sclerosis 
are due to a defect of vital endurance, or, as Gowers has recently termed 
it, to an " abiotrophy.'' Both of these diseases come on, as a rule, early 
in life. The optic nerve atrophy appears simultaneously with the spinal- 
cord symptoms, and both manifestations are uniformly progressive. We 
are led to believe, therefore, that we have in the optic-nerve atrophy the 
same primary cause — a lack of vital endurance. It is difficult to ex- 
plain, however, why the optic nerve or certain tracts of the spinal cord 
should be picked out for degeneration. 

Lack of vital endurance may be manifested late in life, as, for in- 
stance, in such diseases as paralysis agitans. It is possible that this 
explains the unilateral slowly progressive ascending paralysis described 
by Charles K Mills. 

Such degenerations as are due to pernicious anaemia are undoubtedly 
toxic in origin. The poison which is generated by the disease circulates 
in the blood and acts upon the nervous structures. It may be due to a 
lack of resisting power that the spinal cord and the optic nerves are 
affected. 

Tabes dorsalis is also a disease which is produced by a toxin, and 
it is most probable that posterolateral sclerosis, which comes on late in 
life, is due to a similar cause — a syphilitic toxin — and that the cause of 
the optic-nerve atrophy in the two affections can be similarly explained. 

It is noticeable that in all diseases just mentioned, the optic-nerve 
atrophy is progressive, and that return of function does not occur. Oph- 
thalmoscopically, we find a gray degeneration of the disk. The retina, 
as a rule, is not affected. There is usually a peripheral restriction of 
the fields, and there may be a central scotoma. 



580 THE EYE AND NERVOUS SYSTEM 

The pupillary phenomena are attributed to the disturbance of the 
cervical sympathetic. This will be later fully discussed. 

PRIMARY DEGENERATION OF THE PYRAMIDAL TRACTS 
(PRIMARY LATERAL SCLEROSIS). 

In this affection both pyramidal tracts are diseased, the nerve cells 
in the anterior horns of the spinal cord being normal. Both lower limbs 
are usually involved, and we have the well-known picture of lateral 
sclerosis. The limbs are weak and very spastic. All the tendon reflexes 
are increased, and walking is usually impossible because of the weakness 
and the contractures. There is no atrophy of the muscles. As the 
disease progresses, the upper limbs become involved similarly to the 
lower. 

As a rule, there are no eye symptoms; but, rarely, optic atrophy 
similar in character to that seen in tabes is observed. Gowers mentions 
a case of spastic paraplegia in a boy of twenty, who, besides the spinal- 
cord symptoms, had a gradually developing primary optic atrophy. 
The acuity of vision was reduced to one-tenth. The fields presented a 
peripheral restriction. There was a well-marked central scotoma for red 
and green, which was irregular in outline, but extended to an average 
of about ten degrees from the fixing-point, and, in one eye, to twenty 
degrees. 

The degeneration in the pyramidal tracts and in the optic nerves 
was simultaneous and progressive in character, and argued for the 
abiotic nature of the disease. Again, the eye symptoms were similar to 
those seen in an ordinary atrophy of spinal origin. Optic neuritis is 
said to have occurred in the course of lateral sclerosis, but an examina- 
tion of the recorded cases fails to show clearly such an occurrence. 

AMYOTROPHIC LATERAL SCLEROSIS. 

In this form of primary disease of the motor system there is, in 
addition to the clinical picture of lateral sclerosis just described, an 
atrophy of the muscles of the affected limbs. This is caused by the 
involvement of the nerve-cells of the anterior horns in the spinal cord. 
The disease is progressive, and finally involves the different nuclei in 
the medulla oblongata and pons, giving, besides the spastic symptoms, 
bulbar phenomena, such as difficulty in speech and deglutition, drib- 
bling of saliva, atrophy of the tongue, limbs, and face, and fibrillary 
twitching of the parts involved. 

If the disease is limited to the spinal cord, eye symptoms are 
rare. When the bulb becomes involved, there* may be any of the ocular 






POSTEROLATERAL SCLEEOSIS 581 

phenomena common in bulbar palsy, which will be described in another 
chapter. 

Irregularity of the pupils, with partial or total loss of reaction to 
light, and optic atrophy have been described. The pupillary phenomena 
are explained by the sympathetic involvement; while the optic-nerve 
atrophy is similar to that seen in other primary degenerations of the 
pyramidal tracts. Spiller mentions a weakness of one external rectus 
muscle in one instance. 

PROGRESSIVE ASCENDING UNILATERAL PARALYSIS. 

This disease, which has only recently been described by Mills, is 
a form of primary degeneration of one pyramidal tract, ascending in 
character. A lower or an upper limb is first affected; then, in the 
course of some time (in the original case, eighteen months), the other 
limb is involved, giving the picture of a hemiplegia. A weakness of 
the lower distribution of the face is also present. The affected limbs 
are weak and spastic, and the reflexes are increased. 

In a case subsequently described by Spiller, in which the eye exami- 
nation was made by Dr. Hansell, the report was as follows : 

Right Eye. — Media, clear; fundus, normal. A myopia of 3 D. 
exists. 

Left Eye. — Media, clear ; arteries, small ; veins, normal. The edges 
of the disk are distinct. The nerve-head is white, the atrophy probably 
being spinal in origin. There is no indication of previous neutritis. 

It is interesting to observe that in this case the atrophy of the optic 
nerve corresponded with the primary degeneration of the pyramidal 
tract of the same side, both being on the left ; whereas the other optic 
nerve was normal. 

POSTEROLATERAL SCLEROSIS. 

In this combined system disease there is a degeneration of the pos- 
terior and lateral tracts of the spinal cord. Besides the increase of the 
reflexes, the spastic condition, and the weakness of the limbs that are 
common in lateral sclerosis, there is a spastic-ataxic gait, incoordination 
of the limbs, disturbance of sensation for touch and pain, especially on 
the sole of the foot, in front of the tibia, and on the chest as is common 
in tabes, and a disturbance in the functions of the bladder and the 
rectum. 

Ocular Symptoms. — Rigidity of the pupil to light is unusual, but 
Gowers 15 has observed it in two or three cases. In one instance, accom- 

15 Diseases of the Nervous System, vol. i. 506. 



582 THE EYE AKD NERVOUS SYSTEM 

modation was lost, the light reaction being normal. It may be said that 
pupillary changes occur in the cases in which syphilitic infection is 
present. Optic-nerve atrophy is rare, although it has been observed in 
a few cases. Usually there is no involvement of the extraocular muscles, 
although nystagmus has been recorded. 

I have had under my care a man about thirty years of age, who, 
besides the symptoms of posterolateral sclerosis, showed, one year after 
the beginning of these symptoms, the following eye phenomena : 

The pupils were three millimetres in width. The direct and con- 
sensual reactions were normal. There was incipient optic-nerve atrophy. 
Both optic nerves were conspicuously pallid and slightly excavated. ~No 
gross fundus changes were observed in the retina or the choroid. On 
lateral deviation to the right or the left, decided nystagmoid movements 
were observed. The examination was made by Dr. E. A. Shumway. 

It is possible that true nystagmus does not occur in this disease, 
but we have what may be called nystagmoid movements, if we choose 
to make such a distinction. In nystagmus the movements may be in 
any direction and to either side of the fixing point, whereas in nystag- 
moid movements the oscillations of the eyeball are always horizontal 
and are obtained only on lateral deviation. 

SPINAL-CORD SYMPTOMS IN PERNICIOUS ANEMIA. 

It is well known that we may have changes in the spinal cord due to 
any chronic anaemic condition, and this has been especially well recog- 
nized in pernicious anaemia. Spinal symptoms may appear even before 
the blood-count indicates the disease, but as a rule they are mani- 
fested after the disease has well advanced, or they may come on simul- 
taneously. 

Pathologically, there is a diffuse degeneration of the posterior and 
lateral columns, but the degeneration is not limited to these tracts, and 
extends beyond these areas to the anterior part of the cord. The process 
is uniform and involves symmetrical areas. 

Besides the usual symptoms of pernicious anaemia, as weakness, de- 
bility, and reduction in the amount of haemoglobin and in the red and 
white blood-cells, the disease usually begins by a feeling of numbness, 
or a pins-and-needles sensation in the legs, followed by weakness and 
spasticity. The reflexes are increased ; in a short time the upper limbs 
become similarly involved, and we have, besides, a considerable ataxia 
of all the limbs and a disturbance of the bladder and rectal functions. 
Later there may be complete loss of sensation, a flaccid condition may 
succeed the spasticity, and all the reflexes may be lost. 



PERNICIOUS ANAEMIA 583 

Ocular Symptoms. — Eye symptoms are rare. Towards the end of 
the affection the pupils have been found unequal, and in one instance 
there was a slight bilateral ptosis and a marked myosis due to sym- 
pathetic involvement. In a few instances reflex iridoplegia has been 
reported. Nystagmus has also been observed. Optic-nerve atrophy is 
extremely rare, only a few cases being on record. 

I have under my observation an interesting case of this kind. The 
patient, a man of 42, without any previous ailment, suddenly had a 
gastric hemorrhage. The following day he began to lose sight in both 
eyes, and in a week he was completely blind, in which condition he has 
remained. He did not experience any weakness in his limbs until one 
day after sight was completely lost, from which time he gradually 
developed the typical spinal symptoms of the affection. The blood- 
count showed the usual changes of pernicious anaemia. 

Ophthalmoscopic examination, by Dr. de Schweinitz, made four 
days after the blindness became complete showed a double optic neuritis. 
The pupils were dilated and were irresponsive. There was shrinking 
of the retinal arteries and overfilling of the veins, and there was some 
oedema of the nerve-head and surrounding area. 

It is hard to explain the evident low-grade optic neuritis which was 
present in this case, as shown by the examination, and the relation it 
bore to the sudden loss of vision. A gastric hemorrhage may bring on 
an atrophy of the optic nerve and cause blindness in a few hours, but it 
will not produce optic neuritis. Most probably there was a low-grade 
optic neuritis present before the hemorrhage came on, but this did not 
seriously affect the vision, and the sudden loss of blood brought on 
the rapid amaurosis. Optic neuritis in the course of pernicious anaemia 
may occur, but it usually gives a picture of an albuminuric retinitis, 
and the low-grade optic neuritis may have been a preliminary stage of 
the affection. Ophthalmoscopical examination at the present, ten 
months after the onset of the disease, shows a typical post-neuritic 
atrophy. There are also nystagmoid movements on lateral deviation. 

INJURIES OF THE SPINAL CORD. 

Injuries to the substance of the spinal cord are by no means rare. 
Eracture and dislocation of the vertebrae are the most common cause. 
Dislocation of the vertebrae is most frequent in the cervical region, be- 
tween the atlas and axis and between the fifth and sixth cervical ver- 
tebrae. The spinal cord may be injured although the vertebrae are not 
at all harmed. There may be at the moment of injury a sudden torsion 
of the cord, which may produce a hemorrhage, or a softening, or both. 



584 THE EYE AND NEKVOUS SYSTEM 

Usually the hemorrhage, or haematomyelia, is in the central part of the 
spinal cord. Direct pressure causes softening of the affected parts, and 
there may he also hemorrhages and areas of softening either above or 
below the point of greatest pressure. Gunshot injuries or stab wounds 
are not unusual. The spinal cord may be directly injured or there 
may be a resulting hemorrhage and softening from injury to the 
vertebrae. 

Symptoms. — The symptoms produced will depend entirely upon the 
part of the spinal cord involved. If there is a fracture or dislocation, 
there is usually a deformity of that part, accompanied by rigidity of the 
back and neck, and pain on movement. All of the limbs are paralyzed 
if the injury is above the cervical enlargement ; if below this part, only 
the lower limbs are paretic. The reflexes are at first lost, and later 
become increased. The rectal and bladder functions are disturbed, and 
sensation is lost, depending upon the area of the cord destroyed. The 
symptoms of traumatic myelitis are identical, except that we have no 
dislocation or fracture of the vertebrae. In central hagmatomyelia the 
same picture is presented, and we may have, besides, the syringomyelic 
disturbance of sensation, and the pain may be excessive. 

We shall here also consider such other diseases which produce symp- 
toms of compression or disturbance of the function of the spinal cord, 
because the ocular phenomena are identical. Such diseases are tumors 
of the vertebrae and of the spinal cord, aneurism of the aorta, caries, 
carcinoma of the vertebrae and the meninges, and pachymeningitis cervi- 
calis hypertrophica. 

Tumors of the vertebrae are far more common than tumors of the 
meninges or of the substance of the cord. Whether the tumor is extra- 
medullary or intramedullary, the symptoms are. usually identical, and 
depend upon the part of the cord involved. The pain in these cases is 
sharper and more persistent than in any other spinal disease, and its 
location depends upon the part of the body supplied by the involved 
spinal segment or spinal root. 

An eroding aneurism of the descending aorta, caries of the vertebrae, 
carcinoma of the vertebrae and of the meninges, and pachymeningitis 
will give symptoms of pressure upon the spinal cord, depending upon 
the part involved, and, as these symptoms in the main resemble each 
other, we will not consider each separately. The differential diagnosis 
can be readily made in most instances by the concomitant symptoms. 

Ocular Symptoms. — There is no subject on which we have less 
accurate information than the ocular symptoms accompanying injuries 
of the spinal cord. Most of the literature consists of a repetition of 



INJURIES OF THE SPINAL COED 585 

statements made by a few authors, and these are by no means definite. 
Considerable confusions exists, partly because the ocular symptoms fol- 
lowing an injury of the spinal cord are confused with the ocular mani- 
festations due to a hysterical or neurasthenic condition following trauma 
of the spine, the so-called traumatic neurosis. 

In the latter condition we may have dimness of vision, pain in the 
head and eyes, reversion of the color-fields, and a concentric diminution 
of the fields of vision, and any of the hysterical phenomena accom- 
panying the disease. This subject will be fully discussed in another 
chapter. 

The ocular phenomena, when they follow a true injury to the spinal 
cord, may be divided into three classes : first, changes in the optic nerve ; 
second, sympathetic phenomena ; and, third, pupillary changes. These 
will be discussed in order. 

Symptoms of Involvement of the Optic-Nerve. — Clifford Allbutt, 16 
in 1870, first called attention to the changes in the fundus oculi in 
injuries to the spinal cord. This author stated that these phenomena, 
which consisted in a slight haziness of the disk and a distention of the 
retinal veins, did not occur except in chronic cases, and only in the 
course of a few weeks or months, and that the higher the lesion the more 
likely are these symptoms to occur. These findings are somewhat at 
variance with later reports. Thorburn recorded three cases of injury to 
the cervical cord with ophthalmoscopical changes, but in his cases the 
symptoms appeared in the acute and not in the chronic cases. Taylor 
and Collier 17 record two cases of fracture and dislocation. In one 
case there was a definite optic neuritis in one eye, apparently caused by 
a piece of bone pressing upon the lower cervical cord. When this was 
removed the neuritis rapidly disappeared. 

The early report of Allbutt is by no means definite, as we are not 
told how often and when the eye examinations w T ere made. Again, 
our knowledge at that early period, 1870, was by no means as definite 
as it is at present, and we doubt somewhat the statement that changes 
in the fundus occur only in chronic cases. 

It is probable that at times changes in the disk occur, but these are 
mild and temporary, and, therefore, frequent and early ophthalmo- 
scopical examinations are necessary to detect the condition. Similar 
changes have been described in rare instances in tumors of the cervical 
cord, as in a case of Taylor and Collier, of an extramedullary myxoma 
comprising the third cervical segment. Here there was a double optic 

16 The Lancet, Jan. 15, 1870. ,7 Brain. 1901. 



586 THE EYE AND NERVOUS SYSTEM 

neuritis. In caries of the cervical vertebras we may have slight changes 
in the fundus, but in the cases where there is a definite optic neuritis 
an intracranial tubercle probably is the cause. 

It is curious that no ocular disturbances occur in any case in which 
the lesion is below the second dorsal segment. A few cases are on record 
where lesions below this area produced changes in the fundus, but a 
closer examination always showed involvement of the cervical cord. 
We are led, therefore, to believe that in some cases of injury to the 
spinal cord we may have a low-grade optic neuritis, and that this only 
occurs if the cervical cord is involved. It is difficult to explain this. 
We have seen that optic neuritis occurs in conjunction with myelitis, 
and this has been explained by a similar pathological lesion in the optic 
nerve and in the spinal cord due to the same cause. But here we have a 
traumatic myelitis of the cervical cord, as a result of which there are at 
times mild and sometimes definite changes in the optic nerve. This is 
further shown in the instances in which unilateral compression of the 
cervical cord produced unilateral changes in the fundus. 

Wharton Jones explained this relation by a sympathetic involve- 
ment ; but this is not correct, because in lesion of the cervical sympa- 
thetic we do not have optic neuritis. At present no satisfactory explana- 
tion can be offered ; further study — especially more accurate and fre- 
quent ophthalmoscopic examinations in every case of injury of the 
spinal cord and experimental lesions on lower animals — is necessary 
to solve this interesting problem. 

Symptoms of Sympathetic Involvement. — The cervical sympathetic 
is quite frequently involved in traumatic lesions of the spinal cord. 
Our knowledge of the anatomy and physiology of the sympathetic sys- 
tem is by no means exact. Through experimental lesions upon lower 
animals by Budge and others and by clinical experience, we know that 
in the lower part of the cervical and the upper part of the dorsal cord 
there is a so-called ciliospinal centre. This area has been more accu- 
rately localized in the eighth cervical, and especially in the first dorsal 
segment. The anterior roots of the first dorsal segment seem to contain 
the oculopupillary fibres. This has never been better demonstrated than 
by Oppenheim, who had the opportunity of stimulating with an electric 
current the exposed anterior roots of the first dorsal segment in a case 
of gunshot injury to the spine. Oculopupillary symptoms were present 
on stimulating these roots, while stimulation of the roots below had no 
such result. The eighth cervical root was not exposed. 

The classical symptoms of irritation of the cervical sympathetic 
are enlargement of the pupil, widening of the palpebral angle, a slight 



INJURIES OF THE SPINAL CORD 



587 



exophthalmos, delayed descent of the upper lid on looking downwards, 
paleness of the face, and increase of the sweat secretion. Paralysis of 
the cervical sympathetic produces a small pupil, narrowing of the pal- 
pebral angle, a slight enophthalmus, a warmth or coldness of the face, 
and hypericlrosis or anidrosis. Intraocular tension is somewhat 
diminished. 




Fig. 5.— Case of hemorrhage or traumatic myelitis in the left eighth cervical and first dorsal seg- 
ments, causing Brown-Sequard symptoms, with sympathetic paralysis of the left side, narrowing of 
pupil, narrowing of palpebral fissure, retraction of eyeball, and paralysis of the left hand and fore- 
arm. The photograph shows the patient's inability to firmly close the left hand. (Case of Dr. W. G. 
Spiller, Polyclinic Hospital. ) 

We, however, rarely see all of these symptoms in a lesion of the 
sympathetic. This is partially explained by the different centres in 
the spinal cord for the oculopupillary and the vasomotor, trophic, and 
secretory functions. C. Bernard demonstrated in lower animals that 
by cutting the first dorsal roots oculopupillary phenomena only were 
produced, while cutting the sympathetic fibres coming from the second 
to the fourth thoracic vertebra?, produced vasomotor symptoms. It is 
generally thought that the vasomotor fibres are transmitted from the 
third to the sixth dorsal roots, inclusive. 

Oppenheim's case is proof of this, for there were no vasomotor symp- 



588 THE EYE AND KEKVOUS SYSTEM 

toms on irritating the first dorsal roots. Kraus 18 is of the opinion that 
there are only oculopupillary symptoms if the spinal cord is principally 
affected, but if vasomotor symptoms predominate, the rami communi- 
cantes of the sympathetic are more involved. 

The oculopupillary fibres leave the ciliospinal centre by means of 
the rami communicantes of the first dorsal root and join the inferior 
cervical sympathetic ganglion. Here these fibres are joined by the vaso- 
motor fibres from the second to the sixth thoracic segments, inclusive. 
By means of the cerebral sympathetic, these fibres enter the orbit and 
innervate the dilator pupillse, the non-striped part of the levator palpe- 
brse superioris, the orbital muscle of Miiller, and a small bundle of non- 
stripped muscle which lies behind the globe of the eye and bridges 
across the sphenomaxillary fissure at the back of the orbit. 

In the majority of instances, the only sympathetic phenomena ob- 
served in traumatic lesions of the cervical cord are a myosis, and some- 
times a narrowing of the palpebral fissure. These symptoms are not 
only produced if the lesion is in the eighth cervical and first dorsal seg- 
ments, but as well if the lesion is higher. We do not know the course of 
the fibres of the cervical sympathetic in the upper cervical cord, bulb and 
the brain, but it is probable, as Kocher 1 9 thought, that these fibres run 
together in the eighth cervical segment and then diverge in their course 
cerebralwards. Therefore, a lesion above the eighth cervical segment 
would produce only partial symptoms, as myosis and ptosis, because 
only a number of the sympathetic fibres would be involved. A lesion 
limited to one side of the cord will produce unilateral sympathetic 
symptoms. 

Gunshot injuries of the cervical sympathetic sometimes occur. In 
most of these instances the symptoms produced are those of paralysis 
of the sympathetic accompanied by vasomotor phenomena. Tumors of 
the substance of the cervical cord itself, and extramedullary tumors 
pressing upon the cord or upon the rami communicantes may produce 
sympathetic phenomena. Growths of the glands of the neck, intra- 
thoracic tumors, especially those of the upper posterior mediastinum, 
and aneurisms of the descending aorta, subclavian, common carotid and 
vertebral arteries have also been known to produce symptoms of sym- 
pathetic involvement, but these instances are rare. 

Pupillary Changes. — In a number of instances of injury to the upper 
cervical cord the pupils have been found irresponsive to light, while the 
reaction to accommodation and convergence was normal. The study of 

18 Zeitsch. f. Klin. Med., 1905, vol. xviii. 

19 Die Lasionen d. Ruckenm., 1896. Jena. 



INJURIES OF THE SPINAL CORD 589 

the cause of reflex iridoplegia has very recently taken on an additional 
interest, because of the experiments of Bach and Meyer. 20 While their 
conclusions have been based upon experiments on lower animals, and as 
yet clinical evidence has not substantiated their investigations, still, 
considering our meagre knowledge of the subject, such an important 
contribution should be carefully considered. 

These authors believe that in the upper part of the spinal cord and 
the lowest part of the floor of the fourth ventricle, near the median line, 
there is an inhibition centre for the light reflex, and an inhibitory centre 
for pupillodilation. Irritation of this part always produced pupillary 
rigidity and myosis. A right-sided irritation produced reflex iridoplegia 
upon the left side. Bach also explained the myosis which always accom- 
panied the reflex iridoplegia by a more powerful action of the sphincter 
over the weaker dilator pupilla?, assuming that the irritation of the 
inhibitory light reflex centre had aborted all other irritation in the 
pupil, placing it in a state of absolute rest. 

In a series of cases of tabes and general paresis Reichardt 21 attempts 
to prove that the reflex iridoplegia occurring in these diseases, and also 
in cases of fracture and dislocation of the upper cervical vertebrae, and 
in tumors of that region, are dependent upon the lesion in the spinal 
cord. He examined cases with and without failure of the light reflexes, 
and in every instance where there was pupillary rigidity he found a 
degeneration of the endogenous fibres, especially of the second and third 
cervical segments, in the area between the posterior commissure and the 
ventral part of the column of Goll, the so-called Bechterew's inter- 
mediate zone. 

Reichardt assumes that there are four pupillary centres in the cervi- 
cal cord: First, Budge's centre, the so-called ciliospinal centre in the 
lower cervical cord ; second, a pupillodilator centre in the upper part 
of the cervical cord; third, an inhibition centre for the light reflex; 
and fourth, a centre for dilation of the pupil by irritation of the skin 
covering the cervical region in the fourth and fifth cervical segments. 
Reichardt evidently accepts Bach and Meyer's experiments proving an 
inhibition centre for the light reflex as conclusive. 

This author assumes that the centres two and three, which are 
pupillo-dilators and pupillo-contractors, respond to all kinds of irrita- 
tion, both psychical and sensory, thus causing a constant change in the 
pupil. In destruction of the endogenous fibres of the posterior cord 



20 Arch. f. Ophthalm., vols. lv. and lvi. 

21 Arch. f. Psych, u. Nervenkr., vol. xxxix. No. 1. 



590 THE EYE AND NERVOUS SYSTEM 

there is brought about an abnormal condition and a consequent failure 
of the light reflex. An interruption of the fibres between centres one 
and three will cause the same result, presuming that the course of these 
fibres is in the posterior columns. 

Reichardt does not believe that a traumatic lesion of the upper 
cervical cord produces immediate pupil rigidity, because the endogenous 
fibres would not have time to degenerate, but there probably would be 
loss of the light reflex just before death. In chronic cases, as in a bulbar 
syringomyelia, there would be destruction of these endogenous fibres 
and a consequent pupillary rigidity. The endogenous character of the 
lesion must be shown by an absence of degeneration of the posterior 
roots in the upper dorsal and cervical cords. This author also calls 
attention to the absence of pupillary rigidity in traumatic lesions of the 
upper dorsal and lower cervical cords, for the lesion to produce this sign 
must be proximal to the fourth cervical segment. A number of cases of 
fracture and dislocation and tumors of the cervical cord, with pupillary 
rigidity, are quoted to prove the assertion of the author. 

These statements cannot be definitely accepted without further proof. 
It would be well if in all cases of tabes dorsalis and injury to the upper 
part of the spinal cord, there were a thorough microscopical exami- 
nation made of the uppermost part of the cervical cord, for it is more 
than probable that there is some relation between this region and the 
condition of the pupil and the optic nerve. 

In a case of dislocation of the third cervical vertebras with a result- 
ing myelitis of the fifth cervical segment, the pupils were noted to be 
unequal, and a day before death there was no reaction of the pupils to 
light. Examination of the second, third and fourth cervical segments 
failed to show any degeneration in any part of the sections. 

ACUTE ASCENDING PARALYSIS (LANDRY'S PARALYSIS). 

Considerable confusion still exists a's to the true nature of this dis- 
order, first described by Landry in 1859. According to Landry the 
symptoms begin with a diminution of power in the lower limbs, which 
spreads rapidly and successively involves the muscles of the buttocks, 
loins, abdomen, chest, arms, and of the neck. The limbs are flaccid, the 
reflexes are lost and there may be some paresthetic phenomena, but no 
other sensory disturbances. The bladder and rectum are never involved, 
and the mind remains clear to the end. The course is rapid and may 
be fatal in a few days, either through respiratory or cardiac paralysis. 

This symptom-complex has been modified to a considerable degree, 
and while we still recognize the rapidly ascending form of the paralysis, 



LAKDRY'S PARALYSIS 591 

other symptoms may be present. It can be assumed that the symptoms 
just described are to be found in the early stages of the disorder, and, 
if it be not fatal, we may have added anaesthesia of the limbs, abdomen 
and chest, pain on pressure over the nerve trunks, atrophy of the mus- 
cles, reactions of degeneration, and, sometimes, paresis of the functions 
of the bladder and rectum. If the medulla oblongata becomes involved, 
we have added symptoms of bulbar palsy, as difficulty of speech and 
inability to swallow. 

The etiology of the affection is still obscure. It has been known to 
follow cold, influenza, various infectious diseases, and other similar 
causes. The pathological findings in some cases have been entirely 
negative, but in most of the recent autopsies alterations in the nerve- 
cells of the anterior horns of the spinal cord, and an engorgement of 
the vessels of the cord and the leptomeninges have been found. In some 
instances diffuse inflammation of the spinal cord has been described. 
In most cases a parenchymatous degeneration of the peripheral nerves 
is present, and often these were the only pathological findings, causing 
some authors to believe in the peripheral origin of the symptom- 
complex. 

It is probable that the disorder is due to an acute intoxication, and 
the toxin may be caused by various agents, and this toxin acts upon 
the entire central nervous system. This toxic theory is supported by 
the uniform finding of an enlarged spleen and liver, and the febrile 
symptoms which usually usher in and accompany the paralysis. Bac- 
teriologically a number of bacilli have been found, but none character- 
istic. Buzzard was able to isolate a bacillus from the blood of a patient 
with Landry's palsy which, when cultivated and injected into rabbits, 
caused the symptoms of an acute ascending paralysis. 

Ocular Symptoms. — Eye symptoms in Landry's paralysis are un- 
common, because the affection is usually fatal before the nuclei of the 
eye muscles are involved. Abducens paralysis has been noted. Three 
cases are on record in which ptosis was present. In the case of Hoff- 
mann 22 the patient had difficulty in articulation and in swallowing, 
and the tongue movements were weak. Both facial nerves were pa- 
retic, and there was a right-sided ptosis. Bailey and Ewing 23 recorded 
ptosis in another instance. Goebel, 24 in a brief report, mentions in- 
volvement of all the eye muscles, besides difficulty in swallowing, chew- 
ing, and talking. In these three instances the autopsy showed a diffuse 

22 Arch. f. Psych., vol. xv. 

23 New York Medical Journal, 1896. 
24 Neurolog. Centralbl., 1898, No. 7. 



692 THE EYE AKD NERVOUS SYSTEM 

myelitic process of the medulla oblongata. In Goebel's case, which 
lasted for seventeen days, myelitic degeneration by the . method of 
Marchi was found in the area between the oculomotor nucleus and the 
pyramidal crossing. 

Pupillary symptoms are equally rare. Paresis of accommodation 
has been reported by Bernhardt in one instance, and unequal pupils in 
another. Slow reaction to light may also be present. 

In our present knowledge of Landry's symptom-complex it is diffi- 
cult to give a clear explanation of these ocular disturbances, but it is 
probable that there is a nuclear or fascicular involvement of the 
affected cranial nerves. 

DISEASES OF THE NERVES. 

In this connection we shall consider only the diseases of the brachial 
plexus, because in this type of paralysis the rami communicantes of 
the first dorsal roots may be involved and cause symptoms of sympa- 
thetic paralysis. Vasomotor phenomena are not usually present, be- 
cause the probable course of these fibres is through the .second to the 
sixth thoracic roots. 

Paralysis of the brachial plexus may be total or partial, unilateral 
or bilateral. If total, the arm hangs limply by the side, no movements 
being possible; the muscles are atrophic, electrical reactions of degen- 
eration are obtained, and trophic phenomena are present. Partial 
brachial paralysis may be either of the upper plexus type, the so-called 
Duchenne-Erb form, in which the fifth and sixth cervical roots or the 
fibres in the plexus coming from these roots are involved, or the 
Klumpke, or lower plexus type, in which the eighth cervical and first 
dorsal roots are diseased. 

In the upper plexus form the deltoid, triceps, brachialis anticus, the 
supinator longus and brevis, and the infraspinatus muscles are diseased. 
It is impossible to adduct the arm, and the forearm is extended and 
pronated. Sensation, as a rule, is not disturbed. The muscles are 
atrophic, and there may be reactions of degeneration. In the Klumpke 
paralysis the small muscles of the hand and a number of the muscles of 
the forearm, especially the flexors, are paralyzed. Sensory disturbances 
are common in the hand and forearm, especially in the ulnar distri- 
bution. 

These different types of brachial plexus paralyses are mostly trau- 
matic in origin, and may be due to blows, gunshot or stab wounds, frac- 
ture of the head of the humerus, dislocation of the shoulder, and 
tumors. The so-called obstetrical and narcosis paralyses are included in 






DISEASES OF THE NERVES 593 

the upper-arm type. The disease may also occur idiopathically, the 
cause probably being toxic. 

Ocular Symptoms. — Oculopupillary symptoms, consisting in a nar- 
rowing of the pupil and of the palpebral fissure, can occur only if the 
first dorsal roots are involved, either in the intervertebral foramen, or 
before their separation from the rami communicantes. Therefore, in 
the lower arm type of paralysis, in which the eighth cervical and first 
dorsal roots are diseased, we always have oculopupillary symptoms. It 
is possible, however, to have this type of paralysis without sympathetic 
involvement, if the fibres coming from these roots, in the brachial 
plexus, and not the roots themselves, are diseased. It is difficult, how- 
ever, to make such a clinical differential diagnosis because the symptoms 
are identical, but we can always assume that, if oculopupillary symp- 
toms are present, the first dorsal root is diseased. 

In involvement of the fifth and sixth cervical roots in the inter- 
vertebral foramen, oculopupillary symptoms may occur. Heubner 23 
records three such cases. In the first, there was an osteosarcoma of the 
seventh and eighth cervical vertebra? ; in the second, caries of the 
seventh and first dorsal vertebra?; and, in his third case, there was a 
tumor involving the same area. This can be explained only by the 
accidental involvement of the rami communicantes of the first dorsal 
root, or by involvement of the sympathetic fibres in the spinal cord. In 
disease of the fifth and sixth cervical roots outside of the intervertebral 
foramen, oculopupillary symptoms were noted in a case by Oppenheim, 
in which a tumor of the supraclavicular region pressed upon the fifth 
and sixth cervical roots, and in another similar instance of Levy Dorn's. 
Here there must have been an involvement of the oculopupillary fibres 
in the cervical sympathetic. 

In the Duchenne-Erb type of paralysis, due to a birth palsy, or the 
paralysis occurring in the course of etherization, the traction upon the 
arms may cause an abnormal stretching and tearing of the rami com- 
municantes of the first dorsal root, thus causing sympathetic paralysis 
without the first dorsal root itself being diseased. 

If all of the roots of the brachial plexus are diseased, we may have 
oculopupillary symptoms. As a result of gunshot or stab wounds we 
may have forms of paralysis which do not conform with any of the 
known types, with sympathetic symptoms. In these cases the first dorsal 
roots are either involved, or the oculopupillary fibres in the cervical 
sympathetic are injured. 

25 Charite Annalen.. 1895. 

38 



594 THE EYE AND NERVOUS SYSTEM 

Eulenburg and Guttman do not believe that sympathetic phenomena 
accompany every brachial plexus palsy, but are of the opinion that in 
these cases of traumatic neuritis the inflammation spreads to the spinal 
cord through the involved roots, causing a circumscribed secondary 
myelitis, and that this myelitis occurring in the lowest part of the cervi- 
cal and first dorsal segments causes oculopupillary symptoms. While 
this may be true in some instances, it is not the rule, for experimental 
investigations have demonstrated that cutting the first dorsal roots 
will cause immediate oculopupillary phenomena. 

MULTIPLE NEURITIS. 

Multiple neuritis is produced by a variety of causes, chronic alco- 
holism being the most common. Toxic disturbances, such as are pro- 
duced by the various infectious diseases and the different metallic 
poisons, the chronic diseases such as tuberculosis and malaria, beriberi, 
leprosy and senility, may produce polyneuritis. The disease may come 
on without any apparent cause or follow a cold, it probably being in 
these instances infectious in origin. 

The symptoms of multiple neuritis may develop suddenly or sub- 
acutely. The patient first complains of a feeling of numbness in his 
feet or hands, or of sharp pains, these gradually increasing in severity. 
A gradual weakness in the lower or the upper limbs then appears, but 
sometimes the loss of power may come on very suddenly. There may 
be accompanying febrile symptoms, and the patient may be delirious. 
The muscles are flaccid and the tendon and skin reflexes are abolished. 
There is tenderness and pain on pressure over the nerve trunks and the 
pains may become very acute. Sensation is usually affected, there being 
first hyperesthesia followed by anaesthesia. The paralysis is sym- 
metrical and involves in the lower limbs principally the peroneal and 
anterior tibial nerves, causing bilateral foot drop, and in the upper 
limbs the distribution of the radial, median and ulnar nerves, caus- 
ing wrist drop. The paralysis is degenerative in character and in a 
short time electrical reactions of degeneration are usually obtained. 
The bladder and rectum are hardly ever involved, this being an im- 
portant diagnostic feature. Vasomotor and trophic phenomena are 
sometimes present. 

At times ataxia of the upper and lower limbs is present and the 
patient is unable to walk because of this. This form of multiple 
neuritis is sometimes called peripheral neuro- or pseudo-tabes and it is 
at times impossible to distinguish this disease from tabes dorsalis. We 
have not, however, in the former the chronic onset, the Argyll-Robertson 



MULTIPLE NEURITIS 595 

pupil, the girdle sense and the involvement of the bladder and rectum, 
this being, perhaps, sufficient to make a different diagnosis. 

The cranial nerves are usually not affected in the course of multiple 
neuritis, but instances of multiple neuritis limited to the cranial nerves 
have been noted. The ocular nerves are more often involved than the 
other cranial nerves, especially the sixth nerve. The facial nerves are 
sometimes diseased, especially in conjunction with the sixth nerve; 
more rarely the trigeminus and acoustic nerves are involved. The vagus 
is more commonly affected, especially in severe cases, causing an in- 
crease of the pulse rate, and sometimes death. The involvement of 
the ocular nerves will later be fully discussed. The symptoms above 
described are largely common to all forms of multiple neuritis; the 
special forms will be taken up separately and the ocular symptoms 
accompanying them will be dwelt upon. 

Alcoholic Multiple Neuritis. — The symptoms above described are 
essentially those of alcoholic multiple neuritis. We sometimes have 
in addition a peculiar psychic involvement which consists of loss and 
confusion of memory for recent events, and in addition illusions and 
hallucinations. This is sometimes called Korsakoff's psychosis. 

Pathologically, we have an inflammatory or parenchymatous de- 
generation of the different peripheral nerves, especially of the muscular 
and sensory branches of the skin. It is not uncommon, however, to find 
in this disease an alteration in the cells of the anterior horn and of the 
medulla oblongata and pons. These changes in the nerve-cells consist 
of a displacement of the nucleus to the periphery, and a central chroma- 
tolysis. Diffuse and degenerative inflammations with hemorrhages have 
also been noted, especially in the region of the gray matter of the third 
ventricle and of the aqueduct of Sylvius. This was first described by 
Wernicke, 26 who termed the disease acute hemorrhagic polioencephalitis 
superior and inferior, depending whether the upper or the lower cranial 
nuclei were involved. A degeneration of the anterior and posterior roots 
and of the posterior and anterior horns has also been described. These 
changes, however, are not to be confused with those of multiple neuritis, 
in which we have only alterations in the peripheral nerves. 

Alterations in the muscles are not uncommon. These may consist 
only of a narrowing of the muscle fibres, or they may be more severe, 
causing a loss of striation of the fibres and increase of the nuclei. 
Thomsen 27 described in one case similar alterations in an eye muscle. 

26 Lehrbuch der Gehirnkrankh., vol. ii. 1881. 
27 Arch. f. Psych., 1890. 



596 THE EYE AND NERVOUS SYSTEM 

Ocular Symptoms. — Ocular symptoms are not very rare. We shall 
take up first the alterations in the optic nerve, then the pupillary 
phenomena, and lastly the involvement of the motor cranial nerves of 
the eye. 

Disturbance of sight is uncommon, but when it does occur it is 
generally in the nature of a central scotoma, especially for colors. 
Blindness has been reported in a number of instances, but in none is 
there an uncomplicated history. In the clearest case, that of Render, 28 
the ophthalmoscopic examination was not given, and a number of the 
other cranial nerves were involved. In the other reported cases albumin 
was always found in the urine. 

Optic Nerve. — The optic nerve is very seldom involved in alcoholic 
multiple neuritis. A pathological whitish paling of the temporal side 
of the disk has been described, especially by UhthofT, 29 who examined 
one thousand alcoholics and found such changes in 139. He found, 
by histological examination, a retrobulbar neuritis in six of these in- 
stances. It cannot be assumed, however, that the pathological paling of 
the temporal side of the disk depends upon the retrobulbar neuritis, for 
either condition can occur without the other being present. Optic 
neuritis is an exceedingly rare occurrence. I have been able to find in 
the literature only seven recorded cases. The neuritis may be well 
marked in both optic nerves, or may be limited to one. It is usually 
very mild in character, but may be very severe. The neuritis comes on 
generally late in the disease and in most instances disappears as the 
disease improves. In all of the recorded cases, except in one, a part or 
all of the third, sixth and seventh nerves were also involved. 

I have had under my observation such a case in the wards of the 
Philadelphia Hospital. This man, forty years of age, has been drink- 
ing steadily for the past eighteen years, mostly whiskey, sometimes as 
much as twenty-five drinks a day. He has had delirium tremens once, 
six years ago. He smoked somewhat, but denies venereal history. Four 
months ago he first began to have pain in both eyes and complained of 
photophobia and of a discharge from both eyes. Dimness of vision also 
became apparent in both eyes about the same time. The pain was 
relieved somewhat by the wearing of blue glasses, but sight has grad- 
ually diminished. He never had diplopia. Examination of the eyes by 
Dr. E. A. Shumway was as follows : 

R. V. 5/30. L. V. 5/35. Subacute conjunctivitis in both eyes. 



The American Lancet, 1890. 

Arch. f. Ophthalm., vols, xxxii. and xxxiii. 1887. 



MULTIPLE NEUKITIS 597 

The pupils are equal and respond promptly. The fields are normal, 
No color scotoma. Green is recognized badly. Ophthalmoscopic ex- 
amination. Right eye : Media clear, low grade optic neuritis. Left 
eye : Similar low grade optic neuritis, with fine haze in the vitreous, 
posterior part. 

The patient had dimnution of power in all of his limbs ; the reflexes, 
while not lost, were much diminished ; there was pain on pressure over 
the nerve trunks and spontaneous shooting pains in the limbs. He has 
had previous attacks of loss of power in his lower limbs and has com- 
plained of pain for a long period. The urine examination showed a 
very slight trace of albumin. The neuritic symptoms have improved, 
but the optic neuritis still persists, and sight is no better. 

In these cases we must exclude the occurrence of a large amount 
of albumin in the urine, for a nephritic condition might readily cause 
the optic neuritis. In only one of the recorded cases, that of Thomsen, 30 
was there a pathological examination. Here the examination of the 
optic nerve was not mentioned, although the author states that the 
examination of the brain and the spinal cord was entirely negative and 
that the disease was limited to the peripheral nerves. 

It is difficult to explain why the toxic process caused by the alcohol 
should have a selective action upon certain peripheral nerves, and still 
more difficult to explain why it should involve the optic nerve, which 
is not really a nerve at all. As a rule, intoxications which affect 
the peripheral nerves will not affect the optic nerve. From the in- 
sufficient number of cases on record and the absence of pathological 
and experimental investigations, we cannot assume that the optic 
neuritis is caused by the toxin of alcohol. It is probable that the optic 
neuritis which is present may be due to the chronic meningitis which is 
present in some alcoholics. 

Pupils. — The pupils are usually not affected in alcoholic multiple 
neuritis, but a difference in size is not unusual. I have seen this in a 
number of instances. Uhthoff in an examination of one thousand alco- 
holics found a difference in the size of the pupils in sixty of his cases. 
In twenty-five there was, besides, a slow reaction of the pupil to light, 
and in ten instances there was a reflex pupillary rigidity, the reaction to 
accommodation being nearly always preserved. Oppenheim records 
reflex iridoplegia in both pupils, and in one eye accommodation was 
also lost. Eperon records a similar instance without, however, loss of 
accommodation. Moeli is of the opinion that pupillary rigidity is not 



Neurolog. Centralbl., vol. vi. No. 1. 



598 THE EYE AND NERVOUS SYSTEM 

permanent in alcoholics, it being transient in character. I have also 
observed this in one instance. 

Ocular Nerves. — The eye muscles are not often involved. Unilateral 
external rectus palsy is the most common form of ocular paralysis, but 
both abducens nerves may be paralyzed. The seventh nerve is at times 
also involved, generally with the sixth nerve. In two patients recently 
under my observation, external rectus palsy was present in each, while 
in one the left seventh nerve was also slightly paretic. In one patient, 
hearing was for a time diminished. The fifth nerve is sometimes also 
diseased. Dr. Spiller very kindly furnished me with the records of a 
case of alcoholic polyneuritis in which the exit points of the fifth nerve 
were tender to pressure and the patient complained of a feeling of 
numbness in his face. This disappeared in a short time. 

One or both oculomotor nerves may be involved. More often the 
outer musculature of the oculomotor nerve alone is diseased, causing 
ptosis, while the inner musculature remains free, iritic motion and 
accommodation not being disturbed. Edinger explained this peculiar 
involvement by the theory that the external muscles are paralyzed be- 
cause more strain is thrown upon them by the poison. This, however, 
is not a satisfactory explanation. 

UhthofT, in his one thousand alcoholics, found paralysis of the ocular 
muscles in twenty-two cases ; in four there was transient diplopia, and 
in two bilateral abducens paralysis. The ocular palsies usually appear 
in the height of the disease, in about the third or fourth week, and are 
usually transient. In my cases, the abducens palsy came on in the 
second week and lasted for about two weeks, and then disappeared. In 
one, four weeks afterwards the bilateral abducens palsy reappeared with 
a weakness of the left seventh nerve. Besides, the woman had diffi- 
culty in swallowing and she had the dull, monotonous speech of bulbar 
palsy. The pupils were unequal and wider than normal and reacted to 
light and in accommodation. The neuritic symptoms were still severe. 
In the course of a few weeks the bulbar symptoms disappeared. 

Pathology. — While the number of recorded cases of alcoholic mul- 
tiple neuritis with microscopic examination is large, those with in- 
volvement of the ocular nerves are by no means common. Marina 31 
recorded a case of a man of sixty-one with involvement of the left 
oculomotor and the right abducens and facial nerves, with the symp- 
toms of multiple neuritis. A careful microscopical examination of 
the peripheral nerves and of the brain and spinal cord gave entirely 

31 Ueber multip. Augenlahmung, Leipzig u. Wien, 1896. 



MULTIPLE NEURITIS 599 

negative results. It is well known, however, that this can occur in 
polyneuritis of toxic origin. 

A number of cases are recorded in which the microscopical examina- 
tion showed a peripheral degenerative neuritis of the ocular nerves, 
without an involvement of their nuclei. Such, among others, are the 
cases of Dammront and Mayer, in which there was bilateral external 
ophthalmoplegia, besides an involvement of the seventh, ninth and 
twelfth nerves; of Thomsen, in which there was bilateral abducens 
palsy and ptosis, besides a nystagmus, and a mild left optic neuritis. 




Fig. 6.— Case of alcoholic multiple neuritis with external rectus palsy and bilateral wrist-drop. 
The external rectus palsy is not shown in the photograph. 

In Thomsen's case the only branch of the oculomotor nerve diseased 
was the twig to the levator palpebrse muscle. 

In a later report, Thomsen, 32 in a case of alcoholic polyneuritis with 
ocular symptoms, found the peripheral ocular nerves and their roots 
and nuclei intact, but found hemorrhages in the gray matter of the third 
ventricle, and came to the conclusion that the ocular palsies occurring in 
alcoholic multiple neuritis were due to a cerebral lesion, the polio- 

33 Arch. f. Psych., vol. xxi. Case III. 



600 THE EYE AND NERVOUS SYSTEM 

encephalitis hemorrhagica superior of Wernicke. This observation has 
since been confirmed by others, as Boedeker and Rennert. Cases are 
on record, however, in which, besides this encephalitis, the cranial 
nerves themselves were the seat of a degenerative neuritis (Thomsen 
and Jacobseus). Again, Gudden 33 reported a case of polyneuritis in 
which the encephalopathy process involved the region of the third 
ventricle and the aqueduct of Sylvius without producing any ocular 
symptoms. 

We may, therefore, have in alcoholic multiple neuritis: first, no 
pathological changes either in peripheral ocular nerves or in their cen- 
tral connections ; second, alterations only in the peripheral ocular 
nerves ; third, changes only in the region of the gray matter of the 
third ventricle and the aqueduct of Sylvius ; fourth, a combination of 
the degenerative lesions of the peripheral ocular nerves with the enceph- 
alitic process of Wernicke. 

Clinically, it is almost impossible to differentiate between these 
pathological processes. A rapid onset with a bilateral ophthalmoplegia, 
in which both the outer and the inner musculature are involved, with a 
possible, accompanying optic neuritis, may point to an acute polioen- 
cephalitis. 

Nystagmus has been observed repeatedly in alcoholic polyneuritis, 
especially on lateral deviation. Such cases have been recorded by 
Oppenheim and others. UhthofT, in his one thousand alcoholics, found 
in thirteen, nystagmoid movements on lateral excursion, and in two 
definite nystagmus. Thomsen in a similar examination of a number 
of alcoholics found nystagmus in five. I have observed similar nystag- 
moid movements in a number of my cases, but always on lateral devia- 
tion. External rectus palsy had been present. It has seemed to me 
that these were nystagmoid movements, due to the weakness of the 
abducens nerves and not true nystagmus. In none of the cases of poly- 
neuritis was there nystagmus on direct fixation. 

Lead Multiple Neuritis. — The effects of lead on the central ner- 
vous system are well known. Workers in lead, typesetters and others, 
who come in contact with this metal often suffer from this disease. The 
symptoms of lead neuritis are generally preceded by lead colic. The 
poison seems to have a peculiar affinity for the posterior interosseous, 
causing paralysis of the extensors of the hand and fingers, while the 
supinator longus and triceps muscles are usually spared. The nerves of 
the lower extremity are not, as a rule, involved, but if they are, the 



3i Arch. f. Psych., 1896. 



MULTIPLE XEURITIS 601 

peroneal nerve is usually diseased, the tibialis anticus nearly always 
escaping. Sensation is not often disturbed. Ataxia hardly ever occurs. 
The muscles are atrophic and reactions of degeneration are soon ob- 
tained. A blue line on the gums is an aid to the diagnosis of lead 
palsy. 

In some instances lead poisoning causes intracranial symptoms ; 
this is termed lead encephalopathy. Here we may also have paralysis 
of the cranial nerves and of the vocal cords and the laryngeal muscles. 
All of the ocular nerves, the third, fourth and sixth, may be involved, 
either alone or in combination. Either optic neuritis or atrophy may 
occur. Delirium, coma, convulsions, epileptic seizures, hemorrhages 
and transient hemiplegia have been observed in the course of lead 
encephalopathy. This subject is discussed in another chapter. 

Pathology. — Pathologically, the predilection of the lead poisons for 
the peripheral nerves has been well established. The neuritis is de- 
generative in character. Alterations in the cells of the anterior horn, 
as described by Oppenheim, Spiller and others, may occur, and the 
anterior roots have also been found diseased. Goldflam described 
hemorrhages in the anterior and posterior horns. The muscles are the 
seat of degeneration. 

In the literature there are many reported cases of involvement of 
the cranial nerves in the course of lead multiple neuritis, but in view 
of the scarcity of microscopical examination and our 'insufficient knowl- 
edge of the pathology of lead palsy, there is doubt regarding the exist- 
ence of a pure, peripheral multiple neuritis with involvement of the 
cranial nerves. 

Partial paralysis in the distribution of the v ago-accessory and paraly- 
sis of the trigeminus, facial and the hypogiossus nerves have been re- 
ported, but always with involvement of one or more of the other cranial 
nerves. A mononeuritis of a cranial nerve is not on record. A critical 
analysis always shows, besides, symptoms of cerebral involvement. It 
is striking also that when facial paralysis does occur it is confined only 
to the lower distribution, this arguing for a central lesion. 

A number of cases are noted in which the multiple neuritis was 
said to be confined only to the cranial nerves. Such are the cases of 
Pal, 34 Mannaberg, and Chvostek. 35 

In Pal's case a typesetter of twenty-one had for two years attacks 
of eclampsia, during which she had right-sided abducens and facial 



Ueber multip. Neuritis, Wien, 1801. 
Wiener, klin. Woch., 1898. No. 52. 



602 THE EYE AKD NEKVOUS SYSTEM 

paralysis. After a severe attack of lead colic, disturbances of vision 
appeared, followed by optic neuritis and right-sided abducens and facial 
palsy, with electrical reactions of degeneration. Shortly total amaurosis 
with right-sided ptosis became apparent. There was also ataxia of the 
upper limbs with bilateral, peroneal palsy of the lower extremities. At 
the necropsy the gray matter, especially of the occipital lobes, the 
internal capsule and the medulla oblongata were harder than normal. 
~No mention is made of a microscopic examination of these parts. The 
microscopical examination of the optic and the third, sixth and seventh 
cranial, the radial and peroneal nerves showed partial degeneration. In 
the spinal cord there was a degeneration of the posterior roots and of 
some of the tracts. 

In the two cases reported by Mannaberg there was headache, dizzi- 
ness, bilateral optic neuritis, right-sided facial palsy and right-sided 
neuralgia, limited to the distribution of the fifth nerve in one case, 
while in the other there was bilateral neuroretinitis and right-sided 
facial and abducens palsy. 

In Chvostek's case there was paralysis of both oculomotor, the left 
abducens and the right facial nerves and bilateral optic neuritis. There 
were also severe cerebral symptoms. At the necropsy the brain sub- 
stance was extremely oedematous, the convolutions were flattened and 
the oculomotor and abducens nerves were found degenerated. This 
degeneration was probably due to the pressure caused by the oedema. 

In every one of these cases there were some symptoms of involve- 
ment of the peripheral nerves, besides symptoms of cerebral involvement. 
It is probable that a careful microscopical examination of the brain 
and cord would have shown the alterations found in lead encephalop- 
athy. It is improbable, considering the nature of the poison and its 
widespread effects upon the cerebrospinal system, that the cranial nerves 
should be affected without involvement of the brain substance. We can 
conclude, therefore, that multiple neuritis, limited to the cranial nerves, 
does not occur, and that if in the course of a lead polyneuritis, symptoms 
of involvement of the cranial nerves appear, the brain itself is diseased. 

Optic neuritis alone has been observed repeatedly in the course of 
chronic lead-poisoning. Goldscheider 36 has called attention to the in- 
teresting fact that optic neuritis may occur alone in conditions which 
lead to polyneuritis, as, for instance, in the infectious diseases and in 
the different metallic poisons. It is probable, however, that in these 
cases the optic neuritis is not a precursor of a multiple neuritis, but 

35 Charite Annalen, vol. xvii. 



MULTIPLE KEUKITIS 603 

that it is a manifestation of a cerebral disease. As has been previously 
mentioned, the optic nerves are not, as a rule, affected by the same 
toxic processes that are prone to involve the peripheral nerves. 

Changes in the size of the pupils, with loss of the light reflex, have 
been observed, but these are probably also encephalic symptoms. 

Paralysis of the eye muscles without involvement of the other 
cranial nerves is not very common in lead-polyneuritis. In 1898 Elsch- 
nig 37 collected sixteen cases from the literature. In these the eye 
muscles were either unilaterally or bilaterally involved. In four in- 
stances the oculomotor nerve was totally paralyzed. The trigeminus and 
the abducens nerves were also involved in several instances. There 
was, however, in these cases no clear history, and syphilis could not 
be excluded in several instances. Here also we may assume that these 
paralyses were only a part of the general lead encephalopathy. 

Arsenical Neuritis. — Arsenic is a frequent cause of polyneuritis, 
but I have been unable to find any reference to involvement of any of 
the ocular muscles. Optic neuritis has been reported by Higier, but 
it is probable that there was a cerebral involvement in his case. 

Polyneuritis due to other Metallic Poisons. — Mercury, copper, 
phosphorus, and carbon disulphide have also been known to produce 
multiple neuritis, but these instances are rare. 

The form of polyneuritis due to carbon disulphide is extremely un- 
common and little is known of its pathology, as observations are lack- 
ing. Workers in vulcanized rubber are especially prone to this disease. 
Mental excitation or depression with hysterical manifestations precede 
the neuritis phenomena, which are, as a rule, of the ataxic form and 
resemble greatly the alcoholic type of neuritis. Hysterical symptoms 
are so common that some authors, as Marie, insist that the hysterical 
manifestations are among the most prominent symptoms. 

Ocular phenomena are common and consist, as a rule, of alterations 
in the visual fields, especially for colors. Amblyopia is a fairly con- 
stant symptom. Scotoma either large or small is found in some cases. 
Pupillary rigidity and even nystagmus have been recorded. In this 
country within the year 1905 Jump and Cruice were the first to report 
two such cases, one of which I have had the privilege of studying. 
These cases showed marked hysterical symptoms. The eye examination 
by Dr. Langdon of the first case showed the optic disks to be slightly 
congested with hyperemia of the retinal vessels. The visual fields were 
much contracted. 

37 Wiener, med. Wochen., 1898, Nos. 27-29. 



604 THE EYE AKD NERVOUS SYSTEM 

It is probable that we have here not a pure multiple neuritis but a 
toxic process which so influences the central nervous system as to pro- 
duce the various mental, ocular and neuritic symptoms. It must be 
acknowledged that most of the various manifestations shown in this 
disease are hysterical in nature ; but why should a previously healthy 
individual who is poisoned by carbon disulphide be hysterical only so 
long as the influence of the poison lasts '? 

The influence of toxins, whether generated within or without the 
body, upon the brain and spinal cord is becoming better recognized. 
It is more than probable that their influence is not selective but gen- 
eral, and that we have alterations not only in the peripheral nerves, 
but also in the central nervous system. Such is the case, for instance, 
in lead or alcoholic poisons and in uraemia. 

Carbon Monoxide Neuritis. — Illuminating gas, or carbon monox- 
ide, may produce symptoms which resemble multiple neuritis. Involve- 
ment of the different cranial nerves is not uncommon. Cases are 
recorded in which the third, fourth, fifth, sixth, and seventh nerves were 
paralyzed either wholly or in part. The paralysis persists only for a 
short time and disappears as the general symptoms improve. Blindness 
has also been recorded. Sibelius reported an interesting case of a boy 
of twenty, who, after exposure to carbon monoxide for five minutes, 
became unconscious. He regained consciousness the following day when 
it was found that the boy was blind. The pupils were large and reac- 
tionless and the left seventh nerve was paretic. Subsequently weakness 
of the extremities developed with dementia and epileptic seizures. The 
ocular symptoms persisted until death, which occurred three months 
after. The microscopical examination showed a diffuse encephalitis 
and a disease of the blood-vessel walls, but no thrombosis. The amauro- 
sis w T as explained by a bilateral area of softening in the occipital lobe. 

This case illustrates well that toxic processes do not produce local 
alteration in the peripheral nerves, but that they have a general action 
on the whole central nervous system. 

Sulphonal and Trional Neuritis. — Since the introduction of coal- 
tar derivatives, poisoning by these drugs is becoming known. This is 
especially so with sulphonal. Erbsloh recorded an instance of a man of 
forty who had taken two grammes of sulphonal daily for five days 
and developed symptoms of polyneuritis. Besides, the right pupil was 
larger than the left, the left abducens nerve was paralyzed and he had 
hallucinations of sight. At the necropsy the peripheral nerves were 
found degenerated. Dillingham also records ptosis, which lasted about 
ten days. 



MULTIPLE KEITKITIS 605 

Diphtheritic Paralysis. — Approximately about one-quarter of the 
total number of cases of diphtheria are followed by paralysis. It is 
more liable to follow a severe attack, although paralysis has been known 
to follow a simple sore throat or diphtheritic inflammation elsewhere 
in the body. The older the person the greater is the tendency to 
paralysis. (See Chapter V, p. 231.) 

As a rule, the paralysis does not appear until the diphtheria has 
entirely disappeared, in the third or fourth week and sometimes later, 
although in rare instances it may occur in the height of the disease. 
The symptoms of diphtheritic paralysis will not here be discussed, and 
we shall only consider the ocular phenomena. 

Ocular Symptoms. — Loss of accommodation with impairment of 
vision for near objects, due to paralysis of the ciliary muscles, is the 
most frequent form of paralysis, next to that of the palate. It generally 
follows involvement of the latter or appears at the same time in about 
the third or fourth week. It is the most common of the ocular palsies. 
The ciliary involvement may be so slight that its presence may not be 
detected, especially in children. The patient may not be able to thread 
a needle or read, but may follow the finger. 

The interference with vision will depend upon the normal refracting 
power of the eyeball. In a normal eye near vision will be interfered 
with ; in hypermetropia this will be even more marked, while in a 
myopic eye there may be very little or no appreciable disturbance of 
vision for near objects, for accommodation is unnecessary. 

The pupils, as a rule, are of normal size and the light reflex is never 
lost, although it may be sluggish. Slight contraction of the pupils in 
accommodation may be present, even though accommodation is entirely 
lost, and the former is rarely absent. Loss of accommodation generally 
comes on in two or three days and lasts two or three weeks and 
then disappears. It involves both eyes, but may affect one before the 
other. Ophthalmoscopic examination is always negative. 

Paralysis of the ocular muscles is uncommon. Statistics upon this 
point vary, some authors giving as much as twenty-five per cent, of 
ocular palsies following diphtheria. The external and internal recti 
muscles are most frequently involved. This involvement may be uni- 
lateral, but is commonly bilateral and follows the loss of accommoda- 
tion. The palsy may be so slight as not to be noticed, or may be fluc- 
tuating in character. The paralysis is usually of short duration. 

The oculomotor nerve, either wholly or in part, is rarely involved. 
Ptosis is mentioned only in a few instances. Uhthoff records in a boy 
of ten double external ophthalmoplegia, without involvement of the 



606 



THE EYE AND NERVOUS SYSTEM 



pupils, with a mild ptosis. This lasted a month. Mendel and Gowers 
mention two cases in which almost all of the muscles supplied by the 
oculomotor nerves were affected. In Gowers' case there was also con- 
centric contraction of the visual fields and, according to him, this is not 
so infrequent in diphtheritic palsy, for it generally escapes attention, 
due to the presence of the cycloplegia. 




Fig. 7. — Case of diphtheria, with bilateral external rectus palsy, developing two weeks after the 
subsidence of diphtheritic symptoms, and lasting only two weeks. ( Case from the nervous dispensary 
of the Philadelphia Polyclinic.) 



The prognosis, so far as ocular palsies are concerned, is good. Only 
in very rare instances does the paralysis persist. Gowers mentions two 
cases in which there was a slow, progressive degeneration of certain 
cranial nerves occurring some time after the ordinary palsies appear. 
In one case, besides weakness of the lips, tongue and pharynx, there was 
almost complete internal and external ophthalmoplegia and primary 
atrophy of the optic nerves. In his second case sight began to fail six 
months after the diphtheria and was almost lost at the end of the year. 
Two and a half years after the primary disease, there was gray atrophy 
of the optic disks, left internal ophthalmoplegia, and a weakness of 
some of the movements of both eyeballs, especially upwards. These 
changes were stationary. 

Harris 38 has recently recorded three cases in which bulbar symp- 

38 Brain, vol. xxvi. 



MULTIPLE NEUKITIS 607 

toms followed a simple sore throat. In all of these cases the progress 
of the paralysis was typical of post-diphtheritic palsy. Besides the 
bulbar symptoms, the patient could not close the eyes properly, or early 
in the morning could not open the eyes. The symptoms were sug- 
gestive of myasthenia gravis, but Harris believes that they were diph- 
theritic in origin. 

Pathology. — The pathology of diphtheritic paralysis is still very 
much in doubt, because of the varied findings. As a rule, in the 
majority of cases the changes are confined to a degeneration of the 
peripheral nerves, especially in their distal parts. Usually, the neuritis 
is degenerative, but may be interstitial in character. These changes, 
however, are by no means found in every case. Gaucher records a case 
in which the brain, spinal cord, and peripheral nerves were entirely 
normal, while Hochhaus notes an instance in which the alterations were 
limited entirely to the muscles. Changes in the cells of the anterior 
horns of the spinal cord, hemorrhages in the gray substances of the 
spinal cord, increase of the neuroglia cells, and degeneration of the 
anterior and posterior roots have also been found. 

Changes in the bulbar nuclei with a degenerative neuritis of the 
cranial nerves have been found. Such cases have been recorded by 
Mendel, P. Meyer, and others. In Meyer's case there was a disap- 
pearance of the axis cylinders, changes in the myelin and increase 
of the nuclei of the endoneurium of the third nerve. In one case there 
were minute hemorrhages in the walls of the sixth nerve. 

Thus, from the pathology we cannot come to a definite conclusion 
whether the cause of diphtheritic palsy is central, peripheral, or both. 
We have, however, a definite disturbance of function which is caused by 
a poison circulating in the blood. It is only rational to believe that in 
such a toxic process we may expect alterations in all parts of the anat- 
omy. This is borne out by the pathological findings. It is well known 
that certain poisons have a peculiar predilection for certain parts of 
the nervous system ; in this case it is shown by the loss of accommoda- 
tion. Wilbrand and Saenger believe that the cycloplegia is due to a 
nuclear or central lesion. It is difficult to believe, however, that the 
diphtheritic poison could involve only that portion of the oculomotor 
nucleus which is concerned in innervating the ciliary muscles. It is 
more probable that we have here a peculiar peripheral process which 
involves principally the nerve-fibres, and secondarily the muscles. 

Multiple Neuritis due to Infectious Diseases. — Such infectious dis- 
eases as erysipelas, typhoid fever, pneumonia, measles, scarlet fever, 
gonorrhoea, influenza and rheumatism may cause multiple neuritis, but 



008 THE EYE AND NERVOUS SYSTEM 

these instances are necessarily rare. Eye symptoms are exceedingly 
uncommon. Fuchs, however, records mild grade optic neuritis with 
paling on the temporal side of the disc in a typhoid neuritis. Micro- 
scopic examination showed a retrobulbar neuritis. It is probable that 
the infection did not directly cause the optic neuritis. 

Influenza, or la grippe, may cause various nervous symptoms, among 
which is a mononeuritis of the different cranial nerves. Such are the 
cases recorded by Pfliiger of optic neuritis, by Wilbrand and Saenger, 
Uhthoff and others, of paralysis of the different ocular muscles. Wil- 
brand and Saenger 39 reviewed the literature and collected the following 
cases of ocular paralysis occurring after influenza. In thirteen cases 
the third nerve was involved; in fourteen the abducens, and in nine 
the fourth. In twelve cases there was accommodation paralysis, and 
in one the iris was paralyzed. Ptosis occurred eight times. In five it was 
bilateral, in three unilateral. It is probable that in influenza we have 
a toxic process which acts directly upon the different cranial nerves, 
causing a temporary paralysis. Hemorrhagic neuroretinitis was re- 
corded by v. Ley den. The prognosis, as a rule, is good. 

Malarial Neuritis. — Tropical malaria may cause polyneuritis, but 
this disease is rare in this country. I have seen a number of such cases 
among soldiers in the tropics. Mononeuritis of the cerebral nerves has 
been recorded, and even optic neuritis has been mentioned. In one 
instance bilateral abducens palsy was noted by Strachan. 

Beriberi or Kahlce Neuritis. — This disease is rare in this country, 
although it is prevalent in the sea-coast cities of our Southern States. 
It is quite common in tropical countries. The etiology of beriberi is 
still in doubt, but it is probably caused by soil infection and not by a 
rice diet, although this may be a contributory cause. There are three 
principal forms — the atrophic, dropsical and mixed, these probably 
being different stages of the disease. Besides the ordinary symptoms of 
multiple neuritis there is great debility. Ataxia is almost always 
present. Dropsical effusions in nearly all of the serous cavities may 
be present. 

Eye symptoms occur, but they have been insufficiently studied. 
Kessler examined the eye grounds in sixty cases and found a narrowing 
of the retinal arteries, whitish paling of the papilla and, in a large 
number of cases, the edges of the disk were blurred. It is probable, 
bowever, that these changes in the disk are due to the general nutri- 
tional disturbance which is extreme and not to the multiple neuritis. 



39 Die Neurologie des Auges, vol. i. 248. 



MULTIPLE NEURITIS 609 

Disturbances of vision, even complete blindness, may occur. I have 
seen this in a number of cases among the Filipinos, but this generally 
disappears if the patient improves. The cerebral nerves are sometimes 
involved, as the abducens, facial and hypoglossus. 

Leprous Neuritis. — Both in the nodular and the neuritic types of 
leprosy we have involvement of the eyeball. In the nodular form the 
lepromata may invade the conjunctiva, the cornea, the anterior chamber 
of the eyeball, the iris, or ciliary body, or may originate in these parts. 
These lepromata ulcerate and finally destroy sight in both eyes. 

In the neuritic type, owing to the atrophy of the facial muscles, it 
may be impossible to close the eyes, and there may be eversion of the 
lower eyelid. The upper eyelids may droop. At first there is a con- 
junctivitis with lachrymation, but later in the disease there will be 
ulceration of the cornea, and finally complete destruction of the eyeball. 
It is only late in the disease that such alterations in the eyeball may 
oe expected. I have seen a large number of such cases. 

Tuberculous Neuritis. — This form of multiple neuritis is rare 
and eye symptoms are recorded only in very few cases. Among these 
Uuchs records optic neuritis and nystagmus, and Rosenheim pupillary 
differences. The vagus, facial and phrenic nerves have been affected, 
but not the ocular muscles. 

Syphilitic Multiple Neuritis. — According to Oppenheim, there is 
no doubt of the existence of a multiple neuritis due to the syphilo- 
toxic poison, but its occurrence is doubted by many. Cases are recorded 
in which all of the cranial nerves with the exception of the first were 
involved either wholly or in part. Rudinger reported a very interesting 
case of polyneuritis limited to the cranial nerves which he considered 
as syphilitic in origin. In his case a woman, after exposure to cold, 
began to have paresthesia over the right side of the face and tenderness 
over the exit points of the right fifth nerve. This was followed by a 
paralysis with atrophy of the muscles of mastication of the right side, 
and electrical reactions of degeneration. There was also subluxation of 
the lower jaw and disturbance of taste in the anterior two-thirds of the 
tongue, and a right-sided keratitis neuroparalytica. Neuralgia of the 
cornea preceded the keratitis. The tear secretion in the right eye was 
not disturbed, neither was the sense of smell. The facial nerve was 
paralyzed on the opposite side and the acoustic on the same side. There 
were no signs of a basal meningitis or tumor, but there was a history of 
syphilis. 

Neuritis of one facial nerve has also been reported as occurring in 
syphilis. It is difficult to distinguish, however, these forms from a 
39 



610 



THE EYE AND NERVOUS SYSTEM 



syphilitic meningitis and the occurrence of a syphilitic neuritis alone 
may be well doubted. 

Senile Polyneuritis. — This form of neuritis is characterized by 
the chronicity of its onset, the absence of sensory disturbances and of 
any apparent cause. This disease is rare. Schlesinger mentions a case 





Fig. 8.— Arteriosclerotic neuritis, with paralysis of both external recti, and of the superior and 
inferior oblique muscles of the right eye. (Patient of Dr. C. A. Oliver. ) 

in which there was a transient diplopia and sensory disturbances in the 
distribution of one fifth nerve. 

Von Frankl-Hochwart records an interesting case of this kind. A 
woman of sixty-seven, four years after a stroke of paralysis, noticed a 
dimness of vision in the right eye which was followed in half an hour 
by diplopia. A few hours later the patient could not open the right eye, 
but closure was normal. The eyeball was deviated outwards, the pupil- 
lary light reaction was normal, and there were no changes in the fundus. 
The patient died. The microscopical examination showed some disin- 
tegration of the nerve-fibres of the right third nerve, with great increase 
of round cells between the nerve-bundles. The vessels in the connective 
sheath of the nerve were engorged. The author considered it a case of 
arteriosclerotic multiple neuritis in which, however, only one nerve 
was involved. 

Eranceschi also recorded an instance in which there was paresis of 



MULTIPLE NEURITIS 611 

the third, sixth and seventh nerves of one side. Microscopically diffuse 
areas of softening were found all through the brain. There was a paren- 
chymatous neuritis of the involved cranial nerves. In this case there 
was a sclerotic condition of the arteries, this being the primary cause of 
senile neuritis. 

With Dr. Oliver I have observed an interesting case of arterio- 
sclerotic neuritis in a man of sixty-nine. The patient had been per- 
fectly well up to four years ago when he first complained of diplopia. 
Since then he has complained of occasional headache and of a numb 
feeling in his extremities. The tendon reflexes were not disturbed. 
The pupils were equal and reacted slowly to light and in accommodation. 
The optic nerves were normal. There was paralysis of both external 
recti and of the superior and inferior oblique muscles of the right eye. 
His arteries were markedly sclerotic. There was no history of syphilis. 

There can be no doubt of the occasional occurrence of ocular palsies 
due to the neuritis caused by the pressure of the sclerosed basal arteries. 
This is perhaps not sufficiently recognized as a causal factor. (See 
chapter by Dr. Hirsch.) Pathologically, this condition is sometimes 
observed. Recently I found both optic nerves much degenerated, and 
of crescent shape, due to the pressure of the sclerosed anterior cerebral 
arteries. 

Carcinomatous Multiple Neuritis. — This form of polyneuritis 
may be due first to an autointoxication, second to a carcinomatous infil- 
tration of the sheaths of the peripheral nerves, and, third, to an acute 
general carcinomatous infiltration of the peripheral nerve endings. 

The character of the multiple neuritis due to carcinomatous intox- 
ication does not differ from the other forms of polyneuritis due to toxic 
causes. In a case of Miura's there was a slight optic neuritis with an 
absolute central scotoma. There was also nystagmus on lateral and 
upward deviation and diplopia. These symptoms appeared several 
weeks before death and persisted. The microscopical examination by 
Uhthoff failed to show any cause for the optic neuritis. The various 
peripheral nerves, as well as the orbital parts of the third and sixth 
nerves, showed degeneration. The spinal cord, and the different cranial 
nuclei were normal. The slight swelling of the papillae was attributed 
to the broadening of the nerve-fibres in the upper layers. 

Dr. Spiller and I have recorded a remarkable case of cerebral in- 
volvement due to a cancer. A woman who had a recurrent carcinoma of 
the breast five weeks before death complained of failing visum in both 
eyes, more marked in the left. The right pupil was smaller than the 
left and responded to light and in accommodation, but the left pupil did 



612 



THE EYE AND NEKVOUS SYSTEM 



not. There was some exophthalmos of the right eye. The external 
rectus muscle of each side was paralyzed and there was, besides, a 
paralysis of the left superior rectus muscle, and ptosis on the same side. 
Unfortunately no ophthalmoscopic examination was made. An exami- 
nation several days before death showed an almost complete internal and 
external ophthalmoplegia of the left eye, and a distinct, but not equal, 
weakness of the left seventh nerve. There was some headache and 
dizziness, weakness of the limbs and absent knee-jerks. 




;^' 






Fig. 9. — Case of carcinomatous polyneuritis. Illustration shows marked congestion of the blood- 
vessels and disease of the oculomotor nuclei. 

The macroscopical examination did not show anything. Micro- 
scopical examination showed the optic nerves to be normal. Some of 
the axis cylinders of both third nerves, of the right fourth, of the right 
sixth, and of both seventh nerves, were distinctly swollen, but this was 
especially marked in the right sixth nerve. The left fourth nerve was 
normal. The left sixth nerve was not obtained. Some of the nerve 
cells of both oculomotor nuclei were much diseased, and the nucleus of 
each abducens nerve was intensely diseased. The other cranial nuclei, 
as well as the cortical cells, were normal. The blood-vessels all through 
the brain were intensely congested, — this being especially marked in 
the cerebral peduncles and the posterior part of the pons. 

This case illustrates well the diffuse action of the carcinoma toxin. 
It is also noticeable that the pathological findings are not commensurate 
with the symptoms, for microscopical examination may denote a de- 



MULTIPLE NEURITIS 613 

generation of a cranial nerve, the clinical symptoms of which may have 
been entirely absent and vice versa. 

In the second form of polyneuritis, that due to an infiltration of 
cancer-cells in the nerve sheaths, we may have, as in the case of Saenger, 
an infiltration of cancer-cells in the pia covering the nerve-fibres at their 
exit from the base of the brain. Saenger also found an infiltration of 
cancer-cells in the pial covering of the cortex. 

Raymond has very recently called attention to a third form of 
cancerous polyneuritis, the acute, general, miliary form. The symp- 
tom complex is similar to the other forms of polyneuritis. Macroscopi- 
cally no changes can be found centrally or in the peripheral nerves, but 
microscopic examination shows multiple lesions in the finest intramus- 
cular nerve endings, these being due to the compression by the innu- 
merable cancerous emboli in the muscles, a true carcinomatous neuro- 
myositis. No instance is recorded, however, in which the cranial nerves 
were diseased. 

Diabetic Neuritis. — It is recognized that diabetes may cause a dis- 
ease of the peripheral nerves. It is, however, improbable that the 
occasional involvement of a cranial nerve is part of a peripheral neuritis, 
for when such symptoms occur, we probably have a general toxic process 
due to the diabetic poison. This subject is discussed in another chapter. 

Primary Neurotic Atrophy. — Because of our lack of sufficient 
pathological evidence, confusion still exists as to the cause of this disease. 
It occurs in families and is distinguished by atrophy, developing in the 
distal portions of the extremities. The reflexes are absent and there are 
few sensory disturbances. Pathologically, in the few cases recorded, 
alterations have been found in the peripheral nerves and muscles, and 
the nerve-cells of the anterior horns of the spinal cord. 

Optic atrophy accompanying this disease has been recorded only by 
Ballet, Rose, Yizioli, and A. Gordon. In a case under my observation 
in the Philadelphia Hospital the patient gave a distinct history of 
paralysis of the left external rectus and the left superior oblique muscles. 
The diplopia persisted for some time, but has disappeared. There was 
no history of syphilis. 

It is difficult to explain the occurrence of the optic atrophy and the 
involvement of the ocular muscles. It is probable, however, that we 
have here conditions foreign to the disease itself. 



CHAPTEE XVI. 
LEUKOSES AND PSYCHOSES. 

By G. E. de SCHWEINITZ, A.M., M. D. 

OCULAR MANIFESTATIONS OF HYSTERIA. 

The investigation of the disturbances of visual sensation to which 
hysterics are subject has acquired much importance, and the ocular 
stigmata of hysteria, as Parinaud has said, constitute a symptomatology 
which is distinct and in some phases unvarying. The following some- 
what artificial subdivisions of the subject are convenient: 

1. Complete anaesthesia of the visual sense, or hysterical amaurosis. 

2. Incomplete anaesthesia of the visual sense, or hysterical ambly- 
opia. 

3. Defective and painful visual sensation, or hysterical asthenopia. 

4. Anomalies of accommodation, or hysterical cyclospasm and cyclo- 
plegia. 

5. Anomalies of the iris movements, or hysterical pupil phenomena. 

6. Anomalies of the eyelid and eye-muscle movements, or hysterical 
contractures, pseudopalsies, and palsies of the palpebral and bulbar 
musculature. 

7. Previously unclassified hysterical ocular phenomena. 

I. COMPLETE ANESTHESIA OF THE VISUAL 
SENSE, OR HYSTERICAL AMAUROSIS. 

Hysterical Amaurosis. 1 — This term is applied to a condition char- 
acterized by complete abeyance of visual sensation. It occurs both as 
a unilateral and a bilateral affection. 

(a) Bilateral Hysterical Amaurosis. — The literature has been collected 
and analyzed by Cruchet, 2 Kron, 3 and Kerneis. 4 The last author suc- 
ceeded in gathering 34 cases, and since the publication of his thesis a 
number of additional examples have been reported, — for instance, by 

1 For the historical side of this question and for the earlier observations the 
reader is referred to Gilles de la Tourette's Traite Clinique et Therapeutique de 
l'Hysterie, 1891, p. 321; also to P. Pansier's Les Manifestations Oculaires de 
l'Hysterique, Paris, 1892. 

2 Archiv. de Neurologie, 2d Series, xii., 1901, p. 177. 

8 Neurologisches Centralblatt, xxi. 1902, p. 584. 

* Etude sur la Cecite hysterique ou Amaurose Hyst6rique totale et bilat§rale, 
Bordeaux, 1902. 

614 



NEUROSES AND PSYCHOSES 615 

Vetter, McReynolds, Dupuy-Dutemps, Le Roux, 5 Hirsch, 6 and Ca- 
bannes. 7 

In most instances the loss of sight has been absolute, or, form-sense 
and color-sense being lost, the sensation of bright light is still retained, 
— that is, the patient has subjective sensations of light. The onset may 
be sudden or gradual. Although the conditions necessary to create 
double hysterical blindness usually follow an hysterical attack and the 
affection has been described by Binswanger as an interparoxysmal, 
morbid state, this is not necessarily the case, as the amaurosis has been 
encountered without any connection with an attack, — for example, after 
a surgical operation, or, again, as in Leroux's patient, without any 
preceding hysterical phenomena. Usually both eyes are attacked simul- 
taneously ; sometimes one eye may be affected later than the other, — for 
instance, after a few hours, or even after some months. More women 
than men have been affected (4 men to 22 women, according to Kron). 
The age in women has varied from 10 to 26 years; in men from 22 
to 37 years. The pupils may be dilated or contracted. Generally they 
react to the influence of light; sometimes only a feeble contraction 
follows the light stimulus ; occasionally the pupils are dilated and insen- 
sitive to light (Kerneis). Accompanying the amaurosis the following 
symptoms have been observed in some of the cases: Lagophthalmos, 
ptosis, spasm of the orbicularis, anaesthesia of the conjunctiva, nystag- 
mus, conjugate deviation, and convergent strabismus. 

To establish the diagnosis of hysterical amaurosis there must be 
excluded: Ocular disease sufficiently great to produce blindness, con- 
genital amblyopia with high grades of refractive error, and particularly 
certain forms of so-called retrobulbar neuritis. (Eor further discussion 
of this, see page 638). Evidently complete blindness, in the absence 
of ophthalmoscopic signs of optic nerve disease, associated with normal 
pupil reaction to light impulses, would strongly suggest, if it did not 
prove, the presence of the hysterical taint, but because in rare instances, 
temporarily at least, the pupils under these conditions fail to react to 
light, the absence of the pupil reflex does not necessarily exclude 
hysteria. 

All cases on record have recovered vision sooner or later, and there- 
fore the prognosis quoad visum is good. Indeed, usually when the 
amaurosis disappears, it does so, as Pitres remarks, without leaving 



Archives d'Ophthalmologie, December, 1903.. p. 801. 

Prag. med. Wochenschr., 1904, xii. 288. 

Gaz. hebd. d. sc. med de Bordeaux, 1905, xxvi. 3. 



616 THE EYE AND NEKVOUS SYSTEM 

a trace. The duration of the blindness, however, has varied consid- 
erably. Following Kron's suggestion, the cases may be divided into: 
(a) The transitory forms, lasting from a few hours to several days; (&) 
the short-enduring forms, lasting from two to six weeks; and (c) the 
long-enduring forms, lasting from four months to ten years. To these 
should be added the intermittent varieties described by Koenigstein, in 
which the blindness appears at certain definite hours of each day, and 
lasts for a time and then disappears. According to Kerneis, if the 
blindness has appeared suddenly, it will disappear suddenly ; if it has 
arisen during a crisis, it will be during a crisis that it disappears; if 
it is without a crisis that the blindness has appeared suddenly, it will 
be equally sudden and without a crisis that the patient will recover 
his sight; if it has made its appearance gradually, its disappearance 
will be a gradual one. As Kerneis himself remarks, these rules meet 
with exceptions. The blindness may be exceedingly stubborn and last, 
as has already been noted, for years, even for ten years, as in Harlan's 
well-known case. Before the entire disappearance of the blindness, 
there may be a stage of monocular amaurosis, as in McReynolds's case, 
with recurrences, and occasionally a period of temporary hemianopsia. 
(See also page 636.) The first color seen by the patient on recovery 
from the blindness is red. (Cruchet.) 

Certain writers are sceptical in regard to the long-enduring forms of 
hysterical blindness, — that is to say, sceptical in regard to the hysteri- 
cal nature of the affection. For example, B. Sachs 8 writes : " I would 
prefer not to accept as a fact the occurrence of complete hysterical 
amaurosis of months' duration until more convincing cases shall have 
been brought forward." Similar doubts were entertained by Mauthner, 
and are implied by Schmidt-Rimpler. 9 I have never examined a patient 
with bilateral hysterical amaurosis, except as it occurs in the most tem- 
porary way during an attack, and can reply to these criticisms only by 
saying that some of the cases appear to me to be above suspicion, — for 
example, Mendel's case, which Sachs himself quotes. 

(&) Unilateral Hysterical Amaurosis. — Unilateral hysterical amau- 
rosis, in which there is complete abeyance of the visual sensation of 
one eye, it is usually stated, is a more commonly observed phenomenon 
than the bilateral variety of the affection. According to Kron, however, 
more instances of the double than of the single amaurosis are on record, 
— 26 compared with 23. Doubtless the explanation of this is that all, or 



8 Journal of Nervous and Mental Diseases, xxvi. 1899, p. 347. 

9 Die Erkrankungen des Auges im Zusammenhang mit anderen Krajikheiten, 
Wien, 1898, p. 256. 



NEUROSES AKD PSYCHOSES 617 

nearly all, of the cases of double amaurosis have been recorded, while 
only a comparatively few unilateral instances have been published. 
Either eye may be affected, and, according to Briquet, one eye is not 
more frequently selected than its fellow, but in Kron's collection the 
right eye was blind 13 times and the left 9 times. Females are more 
liable than males, in the proportion of 2 to 1. The affection is not con- 
fined to adults, and pure examples have been observed in children, — for 
instance, by Veasey in a child of 11. I have recorded one case in a 
negro child of 11 and seen another in a girl of 14. The difficulty of 
diagnosis in very young children is apparent. 

Unilateral hysterical amaurosis may arise suddenly as the result 
of emotional excitement, fright, trifling injury, menstrual pain, etc., 
or occur as the termination of an hysterical attack, and return with 
the ending of the second attack, constituting, like the bilateral affection, 
the interparoxysmal morbid ocular condition, or, according to Bins- 
wanger, it may exist for a long time without any connection with 
attacks or emotional disturbance. The period of blindness, like the 
bilateral variety, may be brief, or last for years. One of Pitres's 
patients, a child aged 10, was blind four years. In true unilateral 
hysterical amaurosis the opposite eye possesses normal vision and visual 
field, or even a visual field more extended than that which is ordinarily 
considered normal (Parinaud, Morax). More often the opposite eye 
exhibits defective visual acuity, contracted visual field, etc., symptoms 
which will presently be elaborated (page 621). Under these circum- 
stances there is really bilateral disturbance of the visual sensations, 
— that is, blindnes, or amaurosis, on one side, and reduced vision, or 
amblyopia, on the other. Associated with the amaurosis there may 
be other disturbances of sensation — anaesthesia of the skin, perhaps 
confined to one side, — that is hemiansesthesia, anaesthesia of the 
mucosa of the pharynx, of the conjunctiva, and of the cornea. The 
other ocular conditions previously noted with the bilateral form of the 
affection may also exist. While the amaurotic eye is likely to be on 
the side of the greater cutaneous anaesthesia, this rule is not an inva- 
riable one. (See Page 113.) 

The Effect of Binocular Fixation on Monocular Amaurosis. — 
Schweigger, Bernheim, Pitres, Janet, and other observers have noticed 
and described the disappearance of a monocular amaurosis during 
binocular fixation, and we are especially indebted to Parinaud 10 for a 



10 The Ocular Manifestations of Hysteria, System of Diseases of the Eye, edited 
by Norris and Oliver, vol. iv. 1900, p. 741 ; and Annales d'Oculistique, cxxiv. 1900, 
p. 17. 



618 THE EYE AND NEKVOUS SYSTEM 

study of this subject. Briefly, some hysterics completely amaurotic in 
one eye lose this blindness during binocular fixation, as may be proved 
by ordinary prismatic, stereoscopic, or other tests. Thus, if before the 
admittedly sound eye of a patient completely hysterically amaurotic 
in the other eye a prism is placed through which a test-object is re- 
garded, and two images are seen, there is evidence that the blind eye 
has been associated with the other eye in the act of binocular fixation. 
Eor example, if previous examination has shown that the right eye is 
amaurotic in monocular fixation, while with a prism base down before 
the left eye the patient sees two images, and on closing the right eye 
the lower image disappears, it is evident that the blind eye has par- 
ticipated in the act of binocular vision. 

According to Parinaud, as well as other observers, — for example, 
Gilles de la Tourette, — the re-establishment of vision in the amaurotic 
eye at the moment of binocular fixation is concerned only with the 
central perception. The peripheral portion of the visual field remains 
abolished, both in monocular and binocular vision. Sometimes experi- 
ments fail to elicit the reappearance of sight in the amaurotic eye in 
the act of binocular vision, either because the defective sight is too 
great, or perhaps because the patient never possessed, or possessed only 
in imperfect degree, binocular vision. 

Cruchet 11 describes an amaurotic hysterical patient who did not 
have binocular vision, and who with all the tests answered as if she 
was really blind in one eye. According to him the amaurosis should 
be considered in two stages, — the first one, in which there is complete 
abolition of binocular vision, and the second one, more frequently 
observed than the other, in which there is intermittent and generally 
perverted conservation of binocular vision. He states that in cases 
of unilateral hysterical blindness, monocular vision exclusively by the 
good eye is the rule ; but at certain moments and under certain condi- 
tions the monocular vision of the sound eye becomes monocular vision 
of the amblyopic eye, — that is, there is alternation. Again, monocular 
vision may seem to become binocular, but only apparently. Eeally it 
is simultaneous vision, — that is, fusion of the images does not take 
place. Sometimes, the monocular vision becomes binocular vision ? with 
fusion of certain images and not of others; the binocular vision, to 
use the expression of Antonelli, 12 is dissociated. Finally, at certain 
moments and under certain conditions monocular vision really becomes 



n Archiv de Neurologie, 2d Series, xii. 1901, p. 177. 
"Archives d'Ophthalmologie, xvii. 1897, p. 218. 



LEUKOSES AKD PSYCHOSES 619 

binocular with complete fusion of all the images, a condition which 
Cruchet regards as essentially temporary in character. 

Concerning the Nature of Hysterical Amaurosis. — It may be 
stated in the light of our knowledge that hysterical blindness, such 
as has been described in the preceding paragraphs, is not due to 
any failure of the retina to appreciate the images of external objects, 
but to a functional disturbance of cortical centres. A patient with 
monocular amaurosis of hysterical nature sees with the blind eye, as 
may be determined by the ordinary tests which are used to detect pre- 
tended blindness. 

Again, Binet and Janet have shown that if before the eye of an 
amaurotic hysteric some object is held and the patient is afterwards 
hypnotized, he will be able to tell that which was previously exhibited 
and which he regarded with an apparently blind eye. Evidently the 
patient really saw the object. As Prince puts it, " the hysterically 
blind see, but their visual sensations are not in connection with their 
dominating waking consciousness. There would seem to be a shutting 
off from the field of consciousness of certain perceptions which form a 
group of segmented sensations, more or less by themselves, and which 
by artificial means, — for example, the hypnotic state, — would as memo- 
ries be brought into relation with the remainder of the conscious field." 

Because hysterics apparently blind in one eye may by tests ordi- 
narily applied to malingerers be proven to see with this eye, they have 
been characterized as cheats ; but, as Pitres points out, the uniformity 
of answers given by the subjects of unilateral hysterical amaurosis 
cannot be reconciled with the theory of deception in the gross sense of 
this term. 

According to George C. Harlan, patients with hysterical blindness 
may be divided into three classes : those who deliberately simulate the 
blindness, those who see unconsciously but are not capable of conscious 
vision, and those who are really transiently absolutely blind. 

Binswanger 13 quotes Binet' s conclusion that the disturbances which 
lie at the bottom of hysterical anaesthesia, no matter what its nature may 
be, must have their seat in the cerebral cortex, because in order that a 
peripheral stimulus may produce not only movements but also concepts, 
it must have reached the centres in which concepts are formed, — that is, 
the highest composite centres of the cerebrum. Bernheim believes that 
an amblyopic hysterical subject with the aid of imagination uncon- 



13 Specielle Pathologie unci Therapie. H. Nothnagel, xii. Band. i. Halfte, ii. 
Abth., Die Hysterie. von Prof. Dr. Otto Binswanger, Wien, 1004. p. 070. 



620 THE EYE AND NERVOUS SYSTEM 

sciously neutralizes the visual image which has been perceived in a 
normal manner, although, as has been shown, and as Pitres points out, 
the patient does not neutralize binocular but apparently only monocular 
perceptions. Janet, however, from his experiments leans to the theory 
of Bernheim, and thinks that it demonstrates the psychic or cortical 
nature of this sensory disturbance, while Charcot rejects Bernheim's 
explanation that hysterical blindness is exclusively a psychic phe- 
nomenon, — that is, an illusion of the mind and not a true sensory 
anaesthesia. 

Referring again to the interesting fact that in a certain number of 
instances, at least, monocular hysterical amaurosis disappears in binocu- 
lar fixation, we find that Parinaud 14 dismisses Bernheim's theory that 
such phenomena represent a blindness of the imagination due to the 
destruction of the image by a psychic agent, with the curt expression 
that " he does not understand such language," and thinks that the 
interpretation of hysterical visual symptoms depends upon the belief 
in the existence of certain relations between the retina and the visual 
centres in monocular and binocular sight, as well as definite and distinct 
relations in central and in peripheral vision. He goes on to say, " If 
we admit the crossed relation between each eye and the opposite hemi- 
sphere in monocular vision, the left eye will become amaurotic when the 
right side of the brain is affected ; but if in binocular vision both eyes 
are connected with a single hemisphere, it seems clear that if that 
hemisphere be the left and healthier one, the left and amaurotic eye 
will be able to see." 

Charcot, quoted by Binswanger, has demonstrated that hemianaes- 
thetic patients with monocular color blindness are able to identify the 
color accurately during binocular vision, even with the achromatoptic 
eye, and therefore favors the view that there are functional disturbances 
in the intracerebral centres for monocular vision, those for binocular 
vision being uninjured. Similar observations have been made by Pari- 
naud. I shall return to this subject in a later paragraph. 

While there is no proof of it, so far as I am aware, it is conceivable 
that some toxin is liberated during an hysterical attack and the retina 
poisoned, or perhaps the cortical centres, by this toxin, producing, 
according to its virulence, a temporary, an enduring, or a long-continued 
blindness, exactly as this occurs in uraemia, in diabetes, and in certain 
intestinal fermentations. It would be confessedly difficult to reconcile 
this theory with unilateral amblyopia, unless one were willing to admit 



System of Diseases of the Eye, edited by Norris and Oliver, iv. p. 743. 



LEUKOSES AND PSYCHOSES 621 

a selective action of the toxin upon one retina, exactly as in very rare 
toxic blindness, — for example, quinine blindness, — is unilateral. 

II. INCOMPLETE ANESTHESIA OF THE VISUAL SENSE, OR 
HYSTERICAL AMBLYOPIA. 

This term is applied to a number of different visual disturbances, of 
which the most important are: reduction of visual acuity, contraction 
of the visual field, disturbance of the color and light sense, and anoma- 
lies of accommodation. In contradistinction to the affections which 
have been described in the preceding paragraphs and which represent 
a total loss of visual sense, either unilateral or bilateral, these are incom- 
plete disturbances of sight, They represent, in the language of Bins- 
wanger, the incomplete anaesthesias ; " they are polymorphous and 
composite in character, both as regards their extent and as regards the 
special variety of the visual disturbance." 

(1) Reduction of Visual Acuity. — By this is meant a failure of the 
affected eye, or eyes, to obtain full visual acuity in the absence of 
refractive defects and pathological changes, as not infrequently occurs in 
hysteric patients. 

(2) Disturbances of the Visual Field in Hysteria. — (a) Visual 
Field for White. — Many of the records of the visual field examinations 
in hysterics and neurasthenics are untrustworthy because no definite 
information is given in regard to the light, the size of the test-object, 
character of the refraction, the length of time employed in taking the 
visual field, the distance of the examined eye from the fixation point, 
etc. Unfortunately, there has been no uniform method, and hence the 
comparison between the results of different observers is often difficult 
and probably accounts for the discrepancy in statement. Thus, one 
observer, in taking the visual field, moves the test-object from the 
periphery towards the centre and waits until the patient distinctly sees 
the object, and then records its position on the perimeter arc ; while 
another evidently has recorded the points at which the patient first be- 
comes conscious of some object, — for example, a square of white or 
colored paper on the perimeter arc. There is almost a uniform absence 
of statement in regard to the patient's refraction under these condi- 
tions, and very rarely have control examinations been made with the 
ca::ipimeter, or, except in recent times, with the visual field taken at 
varying fixation distances. (For a further consideration of this sub- 
ject, see Chapter IV.) 

Just as a certain number of hysterics have normal acuity of vision, 
they may also have a normal visual field for white. Pansier 15 states 

15 Les Manifestations oculaires de l'Hysterie, Paris, 1892. p. 17. 



622 THE EYE &KD ]S T EKVOUS SYSTEM 

that although, the visual field for white may be normal, he has found a 
true enlargement of the color field, with or without inversion of the 
color circles, in all non-amblyopic hysterics which he has examined. 
On the other hand, Frankl-Hochwart and A. Topolanski 16 maintain 
that in hysteria with normal sensation there is usually no contraction 
of the visual field, and von Reuss 17 has also noted that hysteria may 
exist without any changes in the fields of vision. John K. Mitchell and 
myself 18 have observed a completely normal character of the visual 
field in a certain number of hysterics, or simply contraction of the color 
fields with unaffected form fields. 

The essential characteristic of the visual field in hysteria is concen- 
tric contraction, if this is measured with a white test-object on a 
black background. It represents, to use the language of Parinaud, " an 
insensibility of a portion of the field to white light, and this insensi- 
bility develops from the circumference to the centre in such a way that a 
graphic drawing of the contraction would be represented by an almost 
circular line." Naturally, as Parinaud further maintains, concentric 
must not be taken in a too literal sense of the term, because, although 
quite noticeable on the temporal side where the field is normally largest, 
it may be most restricted on the nasal side or above. Furthermore, this 
visual field should manifest its contraction at the very beginning of the 
measurement, as von Peuss 19 insists, and not be called into existence by 
fatigue during examination. 

The amount of the reduction of the volume of the visual field varies 
from slight contraction, most noticeable on the temporal side, to such an 
extreme restriction that the most peripheral circle is just beyond the 
fixing point. Binswanger is willing io apply the term " pronounced" 
only to those cases in which the normal visual field is reduced fully 
one-half. Should the patient have possessed originally a minimal or 
small physiological field, slight reduction, if bilateral, would be difficult 
to determine unless one should find, as Parinaud suggests, a concomitant 
hysterical disturbance of accommodation. (See page 28.) Bins- 
wanger has never observed " the gravest cases, in which the visual field 
is reduced to the fixation point," and he therefore differs from Parinaud, 
Gilles de la Tourette, and Janet. I have on a number of occasions seen 
extreme reduction in the size of the visual field (see Fig. 1), and can 
in this respect confirm entirely the observations of the French observers 
concerning which Binswanger is sceptical. 

18 Deutschmann's Beitrage zur Augenheilkunde, ii. 1895, p. 46. 

17 Das Gesichtsfeld bei functionellen Nervenleiden, Wien, 1892. 

18 Journal of Nervous and Mental Diseases, January, 1894. 
19 Loc. cit., p. 52. 



KEITROSES AND PSYCHOSES 



623 



The hysterical field contraction is of long endurance, lasting for 
days, months, and even years, and the variations in size which it under- 
goes, except those which are later recorded, are comparatively unimpor- 
tant until recovery occurs, when the visual field again may assume its 
normal size. 

The behavior of the contracted visual field, when it is measured with 
the point of fixation placed at varying distances from the observed eye, 




Left 



Bight 



Fig. 1.— Grand hysteria, scattered anaesthesia. Woman, aged 26 ; patient of Dr. J. K. Mitchell. Con- 
tracted tubular fields ; partial reversal right ; all color sense absent in left field. 



is of importance. Schmidt-Rimpler 20 maintains that each perimetri- 
cally recorded concentric contraction of the visual field should also be 
investigated with a campimeter stationed at varying distances from 
the patient, and Wollenberg, quoted by Binswanger, found under these 
conditions that in the majority of cases, although not always, as the 
distance between the patient and the blackboard is increased, the size 
of the visual field is enlarged. On the other hand, Richard Greeff 21 
states that the contracted visual field maintains the same size, no matter 
at what distance the point of fixation of the examined eye is placed, — in 
other words, that the field has a tubular character; the hysteric has, to 
use the term which Greeff has given, a " rohrenformiges Gesichtsfeld." 
The examination is made as follows: The visual field is measured, as 
usual, with a perimeter, and if it is found contracted in decided degree, 



^Deutsch. med. Wochenschr., 1892, Nr. 24. 

21 Berlin klin. Wochenschr., May 26, 1902, p. 496. 



624 



THE EYE AJSTD NERVOUS SYSTEM 



the point of fixation for the examined eye is removed one metre distant 
and the measurements repeated. Instead of an increase in the width of 
the field, it may remain just as narrow as it was prior to the lengthening 
of the fixation distance, and the same is true if this is still further 
increased, — for example, to two, three, or five metres. That this observa- 
tion is entirely correct I am able to confirm as the result of many 
examinations. (See Eig. 2.) 

According to Green 7 , the cause of the tubular field is connected with 
the underlying nature of the disease ; that is, in hysterical patients a 
definite conception frequently dominates the sensorium, and this applies 
also to the visual field. Yon Hoesslin is unwilling to admit that symp- 




Left 



Bight 



Fig. 2.— Hysterical girl. Patient of Dr. J. William White. Typical tubular contracted field ; no 
change in size at 30 Cm., 1, 2, 3, and 4 metres. 



toms which are physically impossible are genuine signs of disease, and 
he regards the person who exhibits the tubular field phenomenon as a 
malingerer, and if hysteria is present, then the subject is a hysterical 
malingerer. Arnheim and other observers are not in accord with von 
Hoesslin in this contention, and I agree with them and with Wilbrand, 
that " we have no right to cast suspicion of malingering on a patient 
unless we are able to convict him or her of malingering." 

Although the contraction of the visual field may be extreme, visual 
acuity may be normal ; on the other hand it may be reduced. Inasmuch 
as in many cases the peripheral vision is completely abolished, producing 
typical tubular vision, if central sight is affected then amblyopia may 
give place to amaurosis and the condition of complete hysterical blind- 



LEUKOSES AKD PSYCHOSES 625 

ness. It should be remembered, however, that such amaurosis need not 
have — indeed, does not usually have — concentric visual field contraction 
as its prodrome, but appears suddenly. 

The contraction may be unilateral (8 times among 79 cases examined 
by Parinaud) or bilateral, generally the latter. Usually, as Fere, Pari- 
naud, Landolt, Wilbrand, Saenger, and others have shown, it is asso- 
ciated with disturbances of cutaneous anaesthesia, being, when these are 
present, in the language of von Frankl-Hochwart and Topolanski, a 
fairly constant phenomenon. In bilateral cases the restriction of the vis- 
ual field is usually greater on the hemianaesthetic side, or upon that side 
which exhibits the greater surface or degree of anaesthesia; rarely the 
opposite eye is the more affected. Unilateral contraction of the visual 
field commonly appears on the hemianaesthetic side. It should be re- 
membered, however, that the presence of cutaneous anaesthesia does not 
necessarily imply the presence of restricted visual fields, nor is it neces- 
sary that they should appear, as J. K. Mitchell and myself have shown, 
when the general symptoms of hysteria are of the highest grade. 

According to Gilles de la Tourette, 22 concentric narrowing of the 
visual field is the most frequent ocular stigma in hysterical subjects, 
and Dana says that it is the commonest of all forms of anaesthesia. 
Pitres remarks that it is one of the most frequent sensory disturbances 
of the great neurosis and one of the best stigmata of hysteria. Bins- 
wanger, 23 on the other hand, writes, " The scepticism with which we 
regard the statements of Charcot's school (Galezowski, Parinaud, Lan- 
dolt, Fere, and others) increases in direct proportion to the growth of 
our experience in this subject ; for these investigators attribute too much 
importance to the concentric contraction of the visual field in hysteria." 
The value of this symptom seems to me exceedingly great, although J. 
K. Mitchell and myself were unable to find it as frequently among the 
hysterics whom we examined as would appear to be the case in the 
French clinics. 

It has already been pointed out that the contracted visual field is 
one that appears at once and not as the result of repeated examination, 
and that in a certain sense it is a permanent stigma, or perhaps, more 
accurately, an enduring one, differing in this respect from the one 
which will later be described in connection with neurasthenic cases. It 
is, however, subject to certain variations, which must now be mentioned. 
Thev are these : 



22 Traite Clinique et Therapeutique de l'Hysterie, 1891. 
M Loc. cit., p. 209. 
40 



626 THE EYE AND NERVOUS SYSTEM 

In unilateral visual field contraction the narrowed field may be made 
to disappear by irritating the skin, and to develop in the healthy eye; 
in short, a transfer takes place. It may be transferred to the other 
eye by suggestion. Also, as Parinaud has shown, if the unaffected 
eye be opened and the visual field previously contracted remeasured, it 
may increase its extent. Referring to this fact, Janet 24 suggests that 
under these circumstances the sensations of the sound eye bring into 
personal perception by a pre-established association those of the neg- 
lected eye. The extent of the field is furthermore modified by spherical 
glasses, by tinted glasses, and by the action of mydriatics, as has been 
well shown by Parinaud, Forster, and as I have frequently demon- 
strated. A visual field contracted for a white test-object may reach 
greater or even normal extent if investigated with a bright electric light. 

The visual field may be diminished in size by mental condition, by 
physical depression, preoccupation, emotions, fixed ideas ; in general 
terms, as Janet puts it, in a great number of hysterics the effort of 
visual attention contracts in a very notable way their visual fields. 
The variations in perimetric examinations are not necessarily depen- 
dent upon simple retinal fatigue, but, as Schmidt-Rimpler, Schultze, 
and others have shown, may depend upon a diminution of the cortical, 
psychical functions, and be due to damage of the power of concentration. 

Binswanger emphasizes the importance of the psychic condition at 
the time of examination and its effect on the visual field, and insists 
that patients should be methodically investigated at different times 
with due regard, not only to their power of concentration, but also to 
their emotional state, and to the presence of definite disturbances of the 
intellect, and, finally, to the relation of their condition to antecedent 
hysterical attacks. 

It has been the custom of some authors to classify with hysterical 
fields " the shifting type" originally described by Forster in cases of 
anaesthesia of the retina, in which the visual fields differ according as 
the examination is conducted from the temporal to the nasal, or from 
the nasal to the temporal side, the contraction being pronounced on the 
nasal side in the former and on the temporal side in the latter; the 
exhaustion type of Wilbrand, in which the test-object is moved from 
the temporal to the nasal and from the nasal to the temporal side 
several times in succession, across the entire width of the perimeter, 
indeed, as often as the field continues to diminish ; unstable concentric 



24 The Mental State of Hysterics. Translated by C. R. Corson. G. P. Putnam & 
Sons, 1901, p. 69. 



LEUKOSES AND PSYCHOSES 



627 



limitation, — that is, sl field which is constantly changing during the 
examination; the exhaustion-spiral type, in which the tracing of the 
visual field appears coiled like a watch-spring in consequence of its 
limits becoming concentrically smaller; and finally, the recuperation- 
extension type, in which the restricted field may extend during rest, or 
by a strong effort of will. These five types, classified and elaborated by 
von Reuss, 25 do not, however, belong strictly to pure hysterical cases, 
and will be considered in more detail in the section devoted to neuras- 
thenia. 

One form of field, however, von Reuss feels should be classified 
with those which are more strictly hysterical in type, namely, the 
so-called " oscillating field," described by Wilbrand and 0. Koenig, in 




Fig. 3.— Left field. Oscillating field. (After Wilbrand. ) 

which the object disappears and reappears several times in the same 
meridian, and in which a similar oscillation applies to color. This 
phenomenon has been ascribed to rapid change in the sensitiveness of 
the retina. (Fig. 3.) 

As Wilbrand has shown, if the areas in the visual field in which the 
test-object disappears are studied in association with one another, they 
assume a zonular form, — in other words, an annular scotoma results. 
Such scotomas as hysterical manifestations have been described by a 



Z5 Loc. cit. See also a good review of von Reuss's book by J. J. Evans, Ophthal- 
mic Review, xxi. 1902, p. 339. 



628 



THE EYE AND NERVOUS SYSTEM 



number of observers, — for example, Ziehen and Casey A. Wood. (Eig. 
4.) The latter author, although unwilling to rely upon this sign alone 
in the diagnosis of hysteria, has observed it when other ocular indica- 




Right 
Fig. 4.— Annular scotoma in hysteria ; moderate contraction of form field ; no reversal of color lines. 

tions of this neurosis were wanting. Von Reuss maintains that there 
may be a double-ring scotoma, a fragmentary ring scotoma, or that the 
oscillating field may be associated with central scotoma, and, as in a 
case of Wilbrand's, with scintillating scotoma, or temporary hemi- 







Pig. 5.— Central scotoma in hysteria. 

anopsia. A certain degree of stability characterizes these scotomas 
which distinguishes them from exhaustion cases. According to Wil- 
brand, a central exhaustion scotoma is rare. I have observed an abso- 
lute central scotoma in a hysterical woman which lasted for weeks 
(Fig. 5). 



LEUKOSES AND PSYCHOSES 



629 



(&) Visual Field for Colors. — Under this heading must be considered 
concentric contraction of the color fields, dyschromatopsia and acroma- 
topsia, and certain rare conditions to which the term hyperchromatopsia 
may be given. 

Just as there may be a narrowing of the visual field due to an insen- 
sibility to white light, and which, in the language of Parinaud, consti- 
tutes the fundamental condition of this type of hysterical amblyopia, 
so also there may be an insensibility to colors, which begins at the 
periphery of the visual field and occasions a contraction of the color 
field. The color circles may be found within the narrowed circle for 
white exactly in their physiological order, — that is, from without in- 




Fig. 6.— Right hysterical hemianassthesia ; patient of Dr. Wharton Sinkler. Partial left anaesthesia 
reversal of color lines ; outer circle, white, next inner, red, then blue and green ; form field not con- 
tracted, R. E. ; moderate contraction, L. E. 

ward, blue, yellow, red, green, and violet, — the entire restricted field is 
really a much reduced normal visual field. 

Sometimes, as Nonne, J. K. Mitchell, myself, and others have shown, 
the restriction is confined to the color fields, while the white field 
remains unaffected (Fig. 6, right). As long as the color fields, even 
though restricted, retain their normal physiological relation one to the 
other, such restriction, as Parinaud insists, does not necessarily indicate 
dyschromatopsia, but might depend on individual differences found in 
the normal state, or on the light and the objects employed, and upon the 
narrowing due to insensitiveness to white light. 

Under normal circumstances the color fields when tested with satu- 
rated colors in good illumination occur from the periphery to the centre 
in the following order: blue, yellow, red, green, violet. Now in hys- 
terical patients a somewhat characteristic variation is that the red field is 



630 



THE EYE AND KEKVOUS SYSTEM 



the last to be affected, with the result that its extent may equal that of 
blue, so that these color lines coincide or cross one another, or it may 
exceed it and become the most peripheral of the color circles. 

This is the so-called inversion of the color field, and represents much 
more certainly, again to quote Parinaud, the presence of dyschromatop- 
sia than a mere limitation of the extent of the visual field for colors. 
More than this, the red field may exceed in extent not only the blue field 
as described, but that for white, and become not merely the peripheral 
color circle, but the peripheral circle for the entire field. 

Binswanger states that in his clinic he has observed cases in which 
the visual field for red was extraordinarily diminished, so that it was 
exceeded by the visual field for green; similar observations have been 
recorded by v. Hochwart and Topolanski and Wilder, and Pansier has 




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Bight 



Fig. 7.— Hystero-catalepsy ; woman aged 20 ; patient of Dr. S. Weir Mitchell. Typical concentric 
contraction and reversal of blue and red lines. In some meridians the red field exceeds the white 
field. 

seen the green circle become the most peripheral one. Such exceptions, 
however, do not destroy the main fact, that hysterical subjects have a 
predilection for red, constituting a characteristic of this type of dys- 
chromatopsia (Fig. 7). 

Coming to the question of partial abolition of the color-sense, as 
Landolt has pointed out, this may be confined to the most central por- 
tions of the visual field, — that is, the hysterical patients lose first the 
power of distinguishing violet and green, while they retain their power 
of perceiving the other colors. Gradually, it may be, the ability to recog- 
nize red and orange disappears, while the power to perceive yellow and 
blue persists ; or the perception of red, instead of that of blue, is the 
last to disappear. Therefore Pitres has arranged two series giving the 



NEUROSES AND PSYCHOSES 631 

order in which the power of recognizing the different colors is lost in 
hysterical dyschromatopsia : (1) violet, green, blue, yellow, red, and 
(2) violet, green, red, yellow, blue. In contrast to this, it may be 
pointed out, as Pitres has done, that in the dyschromatopsia of certain or- 
ganic conditions, — for example, chronic retrobulbar neuritis, or so-called 
toxic amblyopia, and tabetic atrophy of the optic nerve, — the perception 
of red is first lost. While most observers agree in stating that violet or 
green is the first color to be lost in hysterical cases, Strtimpell believes 
that a definite order of disappearance, such as has been quoted, is 
entirely too schematic. J. K. Mitchell and myself noted long ago that 
we were not so apt to find in American patients the dyschromatopsia 
which has been described, although we did find the inversion of the 
color field to which full reference has been made, and we also noted 
that the perception of green was more often disturbed than that of the 
other colors (violet was not used in our tests), and in this respect the 
observations coincide with those of Landolt. 

Parinaud 26 has noted the following anomalous conditions : A color 
scotoma having all the characteristics of that seen in toxic amblyopia ; 
a central defect with added peripheral contraction resulting in an annu- 
lar color field in the shape of an intermediary zone between the central 
circle and the peripheral zone ; not only an enlargement of the red field 
exceeding that for white, but a similar enlargement for other colors 
(which Pansier maintains is characteristic, page 9), which were either 
perceived as such or as gray with an intensity greater than that for 
white. I have recently studied with A. G. Thomson an hysterical 
boy with a central color scotoma in his field exactly like that which is 
caused by the abuse of tobacco and alcohol. 

Hysterical dyschromatopsia, or disturbance of color-sense mani- 
fested by inversion of color lines or loss of certain color perceptions, — 
for example, violet and green, — may give rise to or be replaced by hys- 
terical achromatopsia, a word for which we are indebted to Galezowski. 
In this condition, first described by Briquet, the patient is unable to 
distinguish any color, although he recognizes the outlines and shapes of 
objects. Everything appears, as has been well said, like a sketch in 
India ink. To the total achromatoptic the colors have a certain luminous 
intensity, to which brief reference has been made by Obarrio. 27 Inves- 
tigation of an entirely color-blind intelligent female patient demon- 



26 System of Diseases of the Eye, edited by Norris and Oliver, vol. iv, 1900, p. 
736. 

27 Bull, de la Soc. Fr. d'Ophth., vol. xvii. p. 453. 



632 THE EYE AND NERVOUS SYSTEM 

strated that each color matched a certain gray. The patient quickly 
recognized the equivalent gray tones, indeed, more quickly than a normal 
individual. 

Dyschromatopsia and achromatopsia are apt to be associated with 
cutaneous and mucous-membrane anaesthesia. Fere maintains that those 
hysterical patients who have neither narrowing of the visual field nor 
achromatopsia have normal sensibility of the conjunctiva; those who 
have achromatopsia and concentric contraction of the visual field have 
complete conjunctival anaesthesia, and those who have achromatopsia 
and practically abolished visual fields have not only conjunctival but 
also corneal anaesthesia. To these observations Gilles de la Tourette 
ascribes great importance, while Binswanger 28 is unable to confirm the 
existence of such regular interrelation between the sensory disturbances 
of the cornea and of the coverings of the eye on the one hand and defects 
of optical sensation on the other. 

(c) Light-Sense and Color-Sense. — In addition to the concentric con- 
traction of the visual field in hysterical patients, diminution of the 
light- and color-sense may be present, as has been pointed out by Pari- 
naud, Wolffberg, and particularly by von Erankl-Hochwart and Topo- 
lanski. According to the last-named authors, sometimes, although the 
visual field and light-sense may be normal, whether it be accompanied by 
disturbances of cutaneous sensibility or not, the power of perception of 
one or other color — for example, blue — may be defective, and Parinaud 
states that wherever he has found dyschromatopsia it has been accom- 
panied by modification of the normal white perception. He has also 
observed patients who, examined with the photometer, could recognize 
white light only when it was extremely intense, but recognized nothing 
when this intensity was lowered, and yet who had a luminous impression 
when the previously unperceived white surface was viewed through a 
saturated red glass. 

The investigation of the color-sense and light-sense in hysterical 
patients is important, and has not received as much attention as it 
deserves, because if, as Binswanger points out, there should be an agree- 
ment, as there was in von Erankl-Hochwart and Topolanski's examina- 
tions, between the restriction of the visual fields and the reduction of 
the light-sense and color-sense, strong evidence would be present that 
there was no simulation, which from the perimeter findings alone 
might be suspected. Binswanger states that Wilbrand was unable with 
the aid of Eorster's photometer to determine anomalies in the light- 

28 Loc. cit., p. 212. 






XEUROSES AXD PSYCHOSES 



633 



sense in hysterical amblyopia. The investigations of von Frankl-Hoch- 
wart and Topolanski were made with the Chibret instrument. 

(d) Hyperchromatopsia has been referred to and the true enlarge- 
ment of the color fields, to which Pansier ascribes special importance, de- 
scribed. J. K. ^Mitchell and myself have observed an exaggerated 
appreciation of colors with corresponding enlargement of the color 
fields in hysterical hyperesthesia. This observation has been confirmed 
by Erankl-Hochwart and Topolanski (Fig. S). Finally, Binswanger 
has found in cases of cutaneous hyperesthesia and hyperalgia marked 
enlargement of the visual fields for red and green, again confirming the 
observation which Mitchell and I made many years ago. In hysterical 
enlargement of the color fields, the blue may exceed all others and pass 




Left 

Fig. 8.— Hysterical hyperesthesia. Patient of Dr. S. Weir Mitchell. Enlargement of color fields 
and partial reversal of red and blue lines. 

beyond the white, or there may be inversion of the widened color circles 
with red as the most peripheral one ; rarely this position is taken by 
green. 

Concerning the Nature of Hysterical Visual Field Contraction 
and Color-Sense Disturbance. — Although hysterics may have extreme 
contraction of the visual field, they are not inconvenienced by this 
apparent limitation of their peripheral vision, as is a patient, for 
example, with pigmentary retinitis. In a word, their orientation is 
usually perfect. In explanation of this it has been suggested that the 
peripheral visual impressions, although unperceived, bring about in 
the muscular apparatus, to use the language of Parinaud. those in- 
stinctive and unconscious movements by means of which we find our way 
through the external world. Groenouw states that hysterical patients 
with greatly contracted fields find no difficulty in getting about because 



634 THE EYE AND NERVOUS SYSTEM 

the periphery of the retina still retains its power of perceiving large and 
bright objects. Wilbrand explains the phenomenon by assuming that the 
irritability of the retinal periphery is not lost, and that the insufficiency 
of the retina is not so great when the patient is moving about as it is 
during steady fixation. To a certain extent what follows has been dis- 
cussed under hysterical amaurosis. (Page 619.) 

Von Graefe believed that hysterical amblyopia represented an affec- 
tion of the outer layers of the retina and could, in general terms, be 
described as a disturbance of the peripheral optical apparatus. Wil- 
brand has stated that in hysteria certain alterations exist in the visual 
substance of the rods and cones, and that where there was peripheral 
contraction of the visual field with retained central vision, these altera- 
tions were limited to the peripheral retina, but that where the ambly- 
opia was greater, or true amaurosis appeared, they extended and the 
entire retina became involved. This theory seemed to be supported by 
perimetric examinations and the various displacement- and exhaustion- 
types of the field of vision which result from them, the indication being 
that the retina is non-resistant to fatigue. (See also, page 683.) Bins- 
wanger maintains, although he gives full place to the theories which 
have been briefly outlined, that the hysterical contracted field is the 
result of a cortico-sensory fatigue, and of a disturbance of the faculty of 
concentration. 

Pierre Janet 29 regards achromatopsia as consistent with the belief 
that the retina is sound, but that the visual defect is further back in the 
perceptive centre. Charcot also maintained that achromatopsia, and 
therefore dyschromatopsia, are cerebral phenomena. It has been proved 
that a hysterical achromatoptic viewing red on a white ground will see 
only a gray image, but after awhile will obtain a consecutive green 
image. In other words, when the green vibration reaches the per- 
ceptive centre, it is not correctly recognized, but nevertheless acts 
because added to the red vibration it gives the perception of white and 
produces the consecutive green image. 30 Parinaud, investigating the 
color contrasts upon hysterical patients, found that the sensation pro- 
duced by a color that is not perceived can be developed provided the 
complementary color inducing it is normally seen. The converse of 
this, however, is not true. 

Simulation by the Hysterical Visual Field and Hysterical Ambly- 
opia of Organic Defects. — (a) Hysterical Hemianopsia. — There is much 



The Mental States of Hysterics, p. 25. 
Pierre Janet, loc. cit. 



LEUKOSES A^ T D PSYCHOSES 635 

difference of opinion whether hemianopsia occurs as an ocular stigma of 
hysteria, and we have statements varying from those of Parinaud, who 
declares that there is one variety of visual insensibility that hysteria 
appears to be incapable of producing, and that is hemianopsia, to the 
assertion of Rosenthal, 31 that in all cases of hysterical amblyopia which 
he has investigated temporal hemianopsia was present. 

Gowers 32 says that of a large number of cases of hemianopsia which 
have come under his observation one only may have been of hysterical 
origin. He further states, 33 " In the hemianesthesia of hysteria we 
have evidence of arrested action of all the higher special sense-centres in 
one hemisphere. In this association we never meet with the hemianopsia 
that results from disease of the lower centre in the posterior part of the 
hemisphere. Instead, we have crossed amblyopia, as it is termed; 
peripheral vision is lost in both eyes, central vision persists in a small 
area in the opposite eye, and in a larger area in the eye of the same 
side. No trace is found of any relation to the half fields." 

Hemianopsia has been reported by Westphal, Galezowski, Svynos, 
Bonnefoy, M. W. Zimmerman, Henry Lloyd, Dejerine, Koenig, and a 
number of other observers. 34 

Dejerine and Vialet, 35 referring especially to neurasthenia and trau- 
matic neurosis, describe a special form of hemianopsia which they think 
constitutes an ocular symptom of the same order as restriction of the 
visual field, and which is distinguished from the hemianopsia of organic 
origin by the variability of the limits of the preserved half field, and 
which may last for years. It is difficult to resist the belief that this 
represents rather an unusual and irregular contraction of the visual 
field, with a much greater restriction on one side .than on the other, 
than a true hemianopsia, and, as far as I am able to judge from an 
examination of the literature, hemianopsia as an enduring ocular symp- 
tom of hysteria, in the same sense as concentric contraction of the visual 
field, does not exist. 

On the other hand, it must be admitted that as a temporary phe- 
nomenon it has been observed a good many times by those who are 

31 Wiener med. Presse, Nr. 23, 1879, p. 736. In Gilles de la Tourette's book 
Rosenthal is quoted as having written a letter to Charcot in which he appears to 
retract the statement just quoted, and says that hysterical patients exhibit only 
amblyopia and concentric contraction of the visual field, and not hemianopsia. 

32 Diseases of the Nervous System, American edition, 1888, p. 584. 

33 Clinical Lectures, Second Series, Subjective Sensations, 1904. p. 11. 

34 Consult Wilfred Harris, Brain, 1897, p. 308; also Koenig, Rec. d'Oph., 
3 Series, 22, 1900, p. 397. 

35 Annates d'Oculistique, 1894, tome cxii. 



636 



THE EYE AND XEEVOUS SYSTEM 



competent to judge, and lias appeared either as a bitemporal defect, a 
homonymous lateral defect, or even, it would seem, as a binasal obscura- 
tion. Furthermore, it would seem from the observations of Pierre 
Janet, 36 who reports a case of transitory hemianopsia which occurred 
in a woman the subject of grand hysteria, and which he believed was not 
brought about by suggestion, that it is possible there may always be a 
period of hemianopsia when the ordinary hysterical amaurosis is grad- 
ually recovering. Indeed, such an observation was made by Galezowski 
and Daguenet long before the publication of Janet's cases. These 
observers found that hysterical amblyopia gradually recovered, and that 
between the period of complete recovery and the period of complete 
amaurosis there was a stage of hemianopsia, and sometimes, instead of 




RigTit 



Fig. 9.— Hysterical girl. Patient of Drs. J. K. Mitchell and G. E. de Schweinitz. Islands of anaesthesia. 
Contracted fields and inversion of color lines on left side where the red exceeds the white field. 

this, a transitory central scotoma. A similar phenomenon was present 
in a patient whose clinical history has been reported by Hirsch in 
Germany, the defect exhibiting itself in a bitemporal form. 

"Whether it is true that in place of a homonymous lateral or bitem- 
poral hemianopsia, as Wilfred Harris has described in his Cambridge 
thesis, binasal hemianopsia may occur I do not know. John K. Mitchell 
and myself published one case in which, somewhat incautiously, we 
made use of the expression that the visual fields had the characters of 
binasal hemianopsia, and since then this case has appeared in literature 
as an example of hysterical binasal hemianopsia. It should be re- 
garded, I feel sure, not in this light, but as an irregular form of con- 

36 La Presse Medicate, 1899, p. 243. 






NEUROSES AND PSYCHOSES 637 

traction with a greater contraction of the field on the nasal side. 
(Fig. 9.) 

According to Gilles de la Tourette, ophthalmic migraine may be 
accompanied by hemianopsia when it is of hysterical origin but it is 
always temporary and may be regarded as an exaggeration of the re- 
striction of the visual field. On the other hand, Babinski 3T doubts 
whether a true relationship between hysteria and an ophthalmic mi- 
graine has been established, and believes that the migraine when asso- 
ciated with the hysteria has an independent origin, making the point- 
that between hysteria and hemianopsia there is no marked relationship, 
while, as he expresses it, the relationship between hemianopsia and 
ophthalmic migraine is like that of parent to child. 

(&) Insular Sclerosis and Hysteria. — Inasmuch as the symptoms of 
hysteria may precede the onset of insular sclerosis or occur during its 
course, this disease may be mistaken for hysteria. Xormally, in the 
visual field between the outer limit of white perception and the outer 
limit of color perception, colors appear light gray, white, or black, and 
according to Head 38 the same phenomenon obtains in the field of the 
hysterical type of disseminated sclerosis, but is not present in the true 
hysteric field. Referring to the blurred vision, or amblyopia, of insular 
sclerosis, Buzzard points out that the optic nerve is diseased, or even 
atrophied, in this disease and normal in hysteria. Other ocular signs 
suggestive of sclerosis are temporary paresis of an ocular muscle, and 
especially nystagmus. (See also, Chapter XI, p. 469.) 

Kampherstein 39 states that regular functional concentric contrac- 
tion of the visual field as it occurs in hysterical amblyopia has not 
occurred in his or in Lubber's investigation of cases of multiple sclerosis. 
According to UhthorT, 40 although the occasional association of visual 
field contraction with insular sclerosis may be explained by a coexisting 
hysteria, it must be remembered that in the former this condition may 
obtain, even in the absence of ophthalmoscopic change, and be none the 
less occasioned by lesion of the opticus. Therefore the diagnosis of pure 
functional disturbance of the visual field under these conditions is 
difficult.. 

(c) Syringomyelia and Hysteria. — Concentric contraction of the visual 
field for all colors has been reported in syringomyelia by Dejerine and 

37 Archiv de Xeurologie, xx. 1890, p. 305. 

38 Quoted by Buzzard, British Medical Journal, Oct. 7. 1893. 
"Archiv f. Augenheilk., Bd. xlix. Erstes Heft. 1903, p. 41. 

*° Graefe-Saemische, Handbueh der gesammten Augenheilkunde, Lief. 68-71, 
1904, p. 352. 



638 THE EYE AND NERVOUS SYSTEM 

Tuilant, but Gilles de la Tourette agrees with Brianceau, whom he 
quotes, that in the majority of cases of this affection the visual field is 
normal, and that when it is contracted it is necessary to search elsewhere 
than in the disease itself for this phenomenon. Hysteria, which may 
accompany the affection, is accordingly given in this respect the etio- 
logical role, in the absence of ophthalmoscopic lesions. (Chapter XV.) 

(d) Exophthalmic Goitre and Hysteria. — Concentric contraction of the 
visual field has been observed in exophthalmic goitre, but in the expe- 
rience of Gilles de la Tourette, under these circumstances hysteria has 
always been associated with the malady. Personally, I have examined a 
great many cases of exophthalmic goitre, and in the absence of ocular 
lesions have not found notable alterations in the visual field, certainly 
nothing similar to those which have been described as more or less 
characteristic of hysteria. (See Chapter XIX.) 

(e) Epilepsy and Hysteria. — It is well known that there may be con- 
traction of the visual field after epileptic attacks, and not only a mere 
contraction of the visual field but an amblyopia, and therefore in these 
respects an analogy between this disease and hysteria is recognized. 
Furthermore, repeated epileptic attacks might lead to an apparent per- 
manent contraction of the visual field or, to speak more accurately, a 
temporary amblyopia which would rapidly subside after one attack 
might persist longer should the attacks be many times repeated. Pari- 
naud, 41 after quoting the observations of Oppenheim and Thomsen, that 
contraction of the visual field is frequently seen in epilepsy, records 
Hitier's examinations in Charcot's clinic, where he found in 87 cases of 
epilepsy a permanent restriction of the visual field in only three. There- 
fore, as there is an occasional more than temporary amblyopia of this 
character, it is necessary that the differential diagnosis of epilepsy and 
hysteria from the visual stand-point should not rest alone upon the phe- 
nomena connected with the field of vision. The other ocular symptoms 
of hysteria should serve to establish the diagnosis. Finally, there is no 
reason why there should not be an association of epilepsy with hysteria, 
an association which has not infrequently been observed. (Chap. XVII.) 

(/) Retrobulbar Neuritis and Hysteria. — It will at once be apparent 
that sudden amaurosis of hysteria and the rapid loss of vision which 
accompanies retrobulbar neuritis on superficial examination bear a 
marked similarity, and there is no doubt that the one has occasionally 
been mistaken for the other. Xaturally, the reaction of the pupil gives 
important information, and if there is blindness with preservation of 

u System of Diseases of the Eye, edited by Norris and Oliver, vol. iv. p. 763. 



NEUROSES AND PSYCHOSES 639 

pupillary contraction to light stimulus, retrobulbar neuritis may be 
excluded. Sometimes, however, as we shall later see, the pupil does not 
react to light, temporarily at least, in hysterical cases, and therefore on 
this point alone a differential diagnosis could not be established, and the 
other symptoms which have been described in connection with hysterical 
amaurosis would almost certainly lead to a proper diagnosis, — for ex- 
ample, the disappearance of monocular blindness in binocular fixation, 
anaesthesia of the conjunctiva, and contraction of the visual field of 
the opposite eye and inversion of the color lines. Symptoms of hys- 
teria not associated with the eye would add most important diagnostic 
information. 

The method in which the blindness occurs in retrobulbar neuritis 
is usually characteristic, inasmuch as the obscuration of vision starts 
in the centre of the field and progresses in from one to eight days to 
complete or nearly complete blindness ; furthermore, there is the 
comparatively rapid replacement of negative ophthalmoscopic signs 
with alterations in the fundus oculi, and pain is evident on movement of 
the eyeball and when it is pressed backward into the orbit. 

Some authors —for example, Bregmann 42 — suggest that just as retro- 
bulbar neuritis may depend upon a primary disturbance of metabolism, 
so also may hysteria, the unknown toxin working on the one hand on 
the optic nerve and on the other on the brain, and think it also possible 
that the optic nerve disturbances may depend upon vasomotor changes 
in the opticus. Hysterical oedema is well known, and therefore it is 
possible that an oedema might arise in the optic nerve which would 
bring about a condition of elevated pressure in the optic-fibres, espe- 
cially in the neighborhood of the optic foramen. 

Not only has restriction of the visual field similar to that seen in 
hysteria been found in the diseases which have been quoted in the pre- 
ceding paragraphs, but in many other organic conditions, — for example, 
brain tumor, lesions of the posterior portion of the internal capsule 
(Charcot), tabes dorsalis, Friedreich's ataxia, and, finally, in certain 
toxaemias, notably those produced by lead, alcohol, mercury, and bi- 
sulphide of carbon. Indeed, for a long time, particularly in France, 
certain toxic agents have been regarded as capable of originating hys- 
teria, and the whole subject has been well elaborated by Groinon. 43 
Sometimes not only is there contraction of the visual field, but reversal of 
the color lines, as in Nieden's case of nitrobenzol poisoning. 44 

42 Zeitschrift f. Augenheilk., vol. v. 1901, p. 391. 

43 Les Agents provocateurs de l'Hysterie, Paris, These, 1889. 

44 Consult The Toxic Amblyopias, by G. E. de Schweinitz, pp. 130, 231. 



640 THE EYE AND NERVOUS SYSTEM 

III. DEFECTIVE AND PAINFUL VISUAL SENSATION, 
OR HYSTERICAL ASTHENOPIA. 

Hysterical Asthenopia. — Thus far the visual phenomena of hys- 
teria have been representatives of various types of anaesthesia of the 
visual sense, or, in other words, a diminished or abolished sensibility to 
visual impressions. In contrast, a number of patients of the hysterical 
type are afflicted with markedly painful visual sensations, notably dread 
of light, lachrymation, blepharospasm, neuralgic pains which appear in 
the neighborhood of the ocular globe and radiate to the temporal and 
zygomatic regions, and imperfect eye endurance. To this set of symp- 
toms the term hyperesthesia of the retina has sometimes been applied. 
They represent the condition which Eorster described as hysterical 
Icopiopia, Donders as painful accommodation, JSTagel as hyperesthesia of 
the ciliary muscle, and Schenkl as hysterical ocular pain, but which, in 
general terms, may be classified under the name attached to the begin- 
ning of this paragraph, — namely, painful visual sensations, or hysterical 
asthenopia. 

Most frequently encountered in women, and not rarely among chil- 
dren, it is probable that the disorders of accommodation and of con- 
vergence which will be described in subsequent pages are always present, 
and are responsible for the photophobia and other symptoms so com- 
monly supposed to indicate hyperesthesia and irritation of the retina. 
Any effort of vision, particularly at close work, is quickly followed by 
ocular pain, brow neuralgia, and diffuse headache, often with micropsia 
and obscuration of vision, and quickly by fatigue. The intolerance of 
light, whether it be diffuse daylight or artificial light, is relieved by 
tinted glasses, to which the patients naturally turn. 

Schwarz believes that to hysterical weakness of sight with reduction 
of visual acuity, differentiated from pronounced hysterical amblyopia, 
should be accredited all the cases which were formerly called " anaes- 
thesia and hyperesthesia of the retina," " amblyopia of the visual field," 
and " nervous astheniopia," but includes with the symptomatology con- 
centric contraction of the visual field, inversion of the color lines, dis- 
turbance of the light- and color-sense, and sometimes central scotoma. 
It would seem, however, that a large majority of these cases present 
only the symptoms before detailed, — namely, painful visual sensations, 
lack of eye endurance, photophobia, chromatophobia, etc. We have in 
these cases, according to Binswanger, to deal with hyperalgesia mani- 
festing itself through light impressions, and he believes that these hyper- 
aigesic conditions form part of a general hyperalgesia, most pronounced 



NEUROSES AND PSYCHOSES 641 

in certain forms of hystero-neurasthenia. I shall, when discussing neu- 
rasthenia, refer to this subject again, as the best types of nervous asthe- 
nopia of this particular variety are seen in the neurasthenic rather than 
the hysteric patient. (See Page 672.) 

Hysterical Ophthalmic Migraine. — At this point reference should 
be made to the association of ophthalmic migraine and hysteria, consti- 
tuting the so-called " hysterical Ophthalmic migraine" described by a 
number of authors. 45 The well-known symptoms of ophthalmic mi- 
graine — paroxysmal headache, hemicranial in type and sometimes pro- 
ceeding to the opposite side, ushered in by visual phenomena, the most 
important of which are various types of expanding and scintillating 
scotomas and hemianopsia, the attack terminating in nausea and vomit- 
ing — occur in hysteria. According to Pansier, the migraine may con- 
stitute the aura of the hysterical attack, or be contemporaneous with it, 
or be accompanied with symptoms which are undeniably hysterical, or, 
finally, be brought into existence by pressure on the migrainous points 
analogous to the hysterogenetic zone, or by suggestion. Whether hemia- 
nopsia may accompany hysterical ophthalmic migraine has been espe- 
cially studied by Babinski and Parinaud, and Gilles de la Tourette, as 
before stated (compare page 637), concludes that if it is present it exists 
only as a temporary phenomenon, or, more accurately, seems to be a 
temporary exaggeration of the concentric restriction of the visual field. 
(Compare Chapter XVII.) 

IV. ANOMALIES OF ACCOMMODATION, OR HYSTER- 
ICAL CYCLOSPASM AND CYCLOPLEGIA. 

It is generally conceded that the most frequent ocular manifestations 
of hysteria are those concerned with anomalies of accommodation, par- 
ticularly in the form of 

(1) Hysterical Accommodation-Spasm, or Cyclospasm. — This 
condition may be associated with amblyopia or occur independently of it, 
and is seen with particular frequency in young subjects. It may be 
partial or total, unilateral or bilateral, and has been particularly studied 
by Parinaud. 46 According to this investigator, hysterical spasm of 
accommodation is similar to that produced by eserin, and has as its 
chief characteristic a displacement towards each other of the pu net ion 

45 Consult Charcot, Legon de Mardi, 1887-1888, p. 101; Babinski, Archives de 
Neurologie, November, 1890; and Fink, Des Rapports de la Migraine ophthalmique 
avec l'Hysterie, These, Paris, 1891. 

46 Annales d'Oculistique, tome lxxix. 1878, p. 218; ibid., tome xcvi. 1880, p. 47; 
see also System of Diseases of the Eye, edited by Norris and Oliver, vol. iv. p. 747. 
" The Ocular Manifestations of Hysteria," by Parinaud. 
41. 



642 THE EYE AND NERVOUS SYSTEM 

proximum and the punctum remotum, — that is to say, there is a diminu- 
tion of accommodation at both ends of the line. It will be observed 
that such phenomena are precisely opposite to those which occur in 
paralysis of accommodation, where the punctum proximum approaches 
the punctum remotum, and when it is coincident with it there is com- 
plete abeyance of the function of accommodation. In the hysterical 
patient, as long as p and r do not coincide, there is some range of accom- 
modation ; when by virtue of the continuance of the displacement of r 
towards p a point is reached where fusion takes place, accommodation 
ceases, and the patient can see only at a fixed point. (See Page- 37.) 

Erom this description of Parinaud's it will be seen that the recession 
of p brings about a state of affairs resembling paresis of accommodation, 
while the advance of r creates an apparent myopia. The former condi- 
tion would be neutralized by a convex glass, the latter by a concave glass. 
In illustration a recent case under my care may be briefly quoted: A 
young hysterical girl, whose refraction was a hypermetropia of 1 D., 
during an attack of spasm of the accommodation required — 1 D. to 
raise her vision to the normal standard, while at the same time her 
amplitude of accommodation was only 3 D., — that is, her near-point for 
fine print was 33 centimetres, which was brought back with a suitable 
convex glass to the normal point, — namely, 12 centimetres. 

As Parinaud points out, the point of fusion may be more or less 
remote from p or from r without losing the character of spasm, and the 
distance of the fusion point from p and r must necessarily be in- 
fluenced by the refractive condition of the eye, — that is, whether the 
eye is myopic, hyperopic, or presbyopic. It will therefore be seen that, 
according to circumstances, — that is, according to whether there has 
been coincidence of p and r, — certain symptoms must obtain, which, to 
repeat, are these : An apparent weakness of accommodation at the near- 
point, a defect of distant vision or a spasmodic myopia, and as a- result of 
the contracture of the ciliary muscle in a manner presently to be ex- 
plained, monocular diplopia, sometimes polyopia, and not infrequently 
micropsia and megalopsia. 

The degree of apparent myopia from cramp of accommodation in 
hysterical patients varies. I have seen in an hysterical woman with 
hypermetropic refraction as follows, +.75s+.50c axis 90, a cramp 
represented by concave 7 D., which restored the visual acuity practically 
to the normal standard. The most remarkable case of this character 
with which I am acquainted is that reported by Loeser. 47 In an hys- 

47 Centralbl. f. prakt. Augenheilk., xxvi. 1902, p. 170. 



NEUROSES AKD PSYCHOSES 643 

terical boy without atropin a vision of 5/10 was obtained with a — 24 D. 
in one eye and a — 22 D. in the other. After atropin the refraction of 
the right eye was + 2c axis 10 and of the left eye + 1.50c axis 25. 

The other symptom to which reference has been made, namely, 
monocular diplopia and polyopia, is not always manifest unless sought 
for by the methods of functional testing, — that is, the patient does not 
necessarily describe its presence. Usually, when the test-object, — for 
example, a small point of light, — is held at a certain distance from the 
eye, it is seen singly, but at points nearer or further from that at which 
single vision is obtained the diplopia appears. According to Parinaud, 
the second image usually separates from the first on the temporal side, 
but a vertical displacement, or one obliquely situated from the test- 
object, is also seen. Occasionally polyopia, or, more accurately, tri- 
plopia, is evident, — that is, a third indistinct image is observed upon 
the opposite side. According to Parinaud, when it appears beyond the 
point of single and distinct vision it can be made to disappear by the 
use of a concave glass, but when it is seen on the proximal side of the 
point of single vision, it disappears if viewed through a convex glass. 

A partial contraction of the muscles of accommodation has been 
described by Borel, Galezowski, and Pansier. This irregular spasm of 
the ciliary muscle creates more or less astigmatism, which disappears 
with the restoration of the patient's general health. 

Schwarz 48 insists, in his description of these phenomena, on the 
difference of their behavior in binocular and monocular vision. Accord- 
ing to him, in binocular vision hysterical accommodation disturbances 
are rare. In high grades of accommodation-spasm there is usually an 
associated convergence-cramp. The cyclospasm appears only during 
fixation, and the object regarded, at first plainly seen, becomes blurred 
and double vision appears. Should this double vision continue, even if 
one eye is covered, the condition is one of monocular diplopia, often 
associated with micropsia. If, on the other hand, the double vision 
disappears when either of the eyes is covered, binocular diplopia exists, 
and there is, if the diplopia is homonymous, hysterical convergence- and 
accommodation-spasm, provided paresis of the abducens is excluded. If 
the double vision is crossed, then there is weakness or paresis of con- 
vergence, provided organic disease is absent. 

Another symptom connected with hysterical spasm of accommodation 
is an inability properly to estimate the size of a test-object which is fixed. 



48 Die Bedeutung der Augenstorungen fiir die Diagnose der Him- und Riicken- 
marks-Krankheiten, Berlin, 1898, p. 80. 



644 THE EYE AND NERVOUS SYSTEM 

Eor example, the test-object appears to grow larger when it is made to 
approach the eye, and appears to grow smaller when it is carried to a 
distant point, corresponding in this respect to the size of the retinal 
images. To this symptom Parinaud has given the name micro-meg alop- 
sia, and he considers it an almost constant one in patients suffering 
from hysterical amblyopia and spasm of accommodation. 

(2) Hysterical Paralysis of Accommodation, or Cycloplegia. — Al- 
though paralysis of the ciliary muscle, which has been observed to exist 
both in a partial and a complete condition, is uncommon as compared 
with spasm of accommodation, it is not unusual to see in young hysterics, 
and especially in hysterical children, mild degrees of accommodative 
weakness, so that the patient is unable to read fine print at the distance 
normal to the age and to the condition of refraction, and is able to do so 
only with the aid of convex glasses. As has already been pointed out, 
this does not necessarily mean that there is a paresis of the ciliary 
muscle, because we have seen that while there may be a recession of the 
near-point there is usually an advance of the far-point, and spasm is 
therefore not excluded, the condition being one of contracture of accom- 
modation. 

On the other hand, in common with many other observers, I have a 
number of times, particularly in mild types of hysterical manifestations, 
often those following injury in young people, observed undoubted 
paresis of accommodation comparable in all particulars to an imperfect 
power of the ciliary muscle, such as might be produced if a cyclo- 
plegic had not exercised its full power. Wilbrand and Saenger have 
recorded instances of paresis of accommodation with transitory diplopia 
in nervous children, and have demonstrated that these temporary condi- 
tions of weakness of accommodation may pass on to paresis of the ciliary 
muscle. They are well known as part of the symptomatology of hys- 
terical asthenopia. (See Page 640.) 

According to Schwarz, 49 hysterical accommodation-paralysis is 
usually bilateral, and generally associated with palsy of convergence. 
If this is present and there exists at the same time no divergent strabis- 
mus at the distant point, the hysterical nature of the disturbance is 
probable. The same author insists, if convergence is normal, that the 
binocular range of accommodation should be tested, after adjustment of 
any difference in refraction of the two eyes. Should, under these cir- 
cumstances, the binocular accommodation range be essentially greater 
than the monocular, the hysterical nature of the paralysis may be as- 

49 Loc. cit., p. 82. 



NEUROSES AND PSYCHOSES 645 

sumed, because with convergence in binocular vision the apparently 
paralyzed accommodation comes into action. If the accommodation in 
binocular vision, in spite of good convergence, remains as much in 
abeyance as in monocular vision, organic paresis is probably present. 
This opinion is disputed by Blok, 50 who reports double hysterical spastic 
mydriasis accompanied by bilateral paralysis of accommodation. So- 
called typical cases of paralysis of accommodation, with diplopia, have 
been recorded by Nonne and Beselin ; they are especially apt to occur if 
the refraction is hyperopic. 

Schwarz's observation of the disappearance of unilateral hysterical 
accommodation-paralysis in binocular vision corresponds to the sub- 
sidence of monocular amaurosis in binocular fixation. In other words, 
the unilateral paralysis of accommodation is apparent only, and Schwarz 
believes that in these cases it has not been proven that the palsy per- 
sisted during binocular vision. Therefore he doubts the occurrence of 
such a pure unilateral hysterical cycloplegia. 

Although the pupils are usually normal in cases of hysterical cyclo- 
plegia, this is not necessarily the case, and mydriasis has been reported, 
— for example, by Donoth, Nonne and Beselin, and Bloq, the last-named 
reporter, as stated above, having recorded a case of mydriasis and 
accommodation-paralysis, at first alternating from the right to the left 
eye, but later becoming stationary in both eyes, and associated with con- 
vergent strabismus. The action of eserin was only slightly manifested. 

Concerning the Pathogenesis of Hysterical Accommodation-Dis- 
turbances. — As has already been sufficiently stated and largely proved 
by Parinaud, the most usual basis of the phenomena under considera- 
tion is a spasm of the ciliary muscle, and this spasm, if complete, 
wipes out the amplitude of accommodation because the eye is adapted 
for a fixed point at which there is single vision. On either side of this 
fixed point diplopia occurs, and the cause of this phenomenon is re- 
ferred by Parinaud to structural defects in the crystalline lens. He 
writes as follows : " The disposition of the anterior portion of the lens 
in three segments favors its changes in curvature, but at the same time, 
owing to their separation from one another by layers of amorphous 
material, each section possesses a focal point of its own, capable of 
producing images distinct from that yielded by the lens as a whole. 
The condition of spasm by increasing the convexity of the anterior 
surface of the lens serves to emphasize the diplopia arising from the 
structural defects described." He believes that in those cases in which 

60 Annales d'Oculistique, cxix. 1898, p. 200. 



646 THE EYE AND NEKVOUS SYSTEM 

anomalies of the central nervous system have been brought forward to 
explain multiple images, the condition of the refraction has not been 
perfectly examined. On the other hand, Pierre Janet, while admitting 
Parinaud's explanations, is unwilling to deny the probable psychic 
origin of some of these phenomena. The probable explanation of 
micropsia and megalopsia has been described, and the interesting 
waking up of accommodative effort (which was in abeyance in monocu- 
lar vision) during binocular fixation has been recorded. 

V. ANOMALIES OF THE IRIS MOVEMENTS, OR 
HYSTERICAL PUPIL PHENOMENA. 

Pupil disturbances of hysterical origin have occasioned much dis- 
pute, and Schwarz's statement that observations concerning anomalies of 
the iris movements of hysterical origin are often inexact is undoubtedly 
true, as is also the remark of Parinaud that pupillary anomalies occur 
rarely as permanent manifestations of ocular hysteria. The following 
conditions have been reported to be present in hysteria : 

(1) Contraction of the pupil, — that is, either spasmodic or paralytic 
myosis. 

(2) Dilatation of the pupil, — that is, either spasmodic or paralytic 
mydriasis, the dilated pupil in one class of cases responding to light im- 
pulses, even when light perception is denied, and in another class of cases 
failing to contract under the influence of light impulse. 

(3) Interchange of mydriasis and myosis. 

(4) Unilateral mydriasis in one class of cases unassociated with 
paresis of accommodation, in another class of cases associated with pare- 
sis of accommodation. 

(5) Dilatation of the pupil with preserved but sluggish reaction to 
light and paralysis of accommodation. 

Contraction of the pupil, or hysterical spasmodic myosis, as an iso- 
lated symptom, if it occurs at all in hysteria, must be exceedingly rare, 
although myotic pupils, according to Westphal, 51 may appear indepen- 
dently of hysterical seizures, or at the conclusion of them, or sometimes 
during trivial attacks. In so-called hysterical pseudomeningitis, myosis 
has been described (Boissard, quoted by Gilles de la Tourette), but 
whether the myosis was spastic or paralytic is a matter of doubt. Hys- 
terical rigidity of the pupil has been observed by Spiller. Dilatation of 
the pupil following myotic contracture has been reported, and Gilles de 
la Tourette considers this preliminary contraction of the pupil im- 

51 Berlin, klin. Wochenschr., xxxiv., 1897, p. 1024. 



LEUKOSES AND PSYCHOSES 647 

portant. He doubts whether there is a true paralysis of the sphincter of 
the iris which causes the subsequent mydriasis;, and attributes the dila- 
tation to a preponderating effect of the dilator after the contracted iris 
has relaxed. The presence of pupil contraction in association with ac- 
commodation and convergence cramp, according to Schwarz, is un- 
doubtedly often present, and Galezowski has noticed myosis as an ac- 
companiment of spasmodic myopia, as it has already been described in 
a previous section, an observation which Parinaud has not been able 
to confirm. In general terms, the myotic pupils, which should be 
ascribed to a spastic condition of the sphincter of the iris, are brought 
about by psychic disturbances, and as already noted, may appear inde- 
pendently of the attacks, or at the conclusion of them ; when the psychic 
disturbance is removed the pupil may dilate. 

Spasmodic mydriasis in connection with hysteria has been described 
frequently. The literature has been reviewed by Aurand and Erenkel, 52 
Eonne and Beselin, 53 Binswanger, 54 and Pichler, 55 and from a study of 
this literature Binswanger asserts that although hysterical mydriasis is 
rare, it has a true and autonomous existence. Clinically it is necessary 
to distinguish between a spasmodic and a paralytic form, either of which 
may be independent, but Binswanger thinks it must be stated that a 
patient may have at the same time spasmodic and paralytic mydriasis. 
We must further grant that in one class of cases of mydriasis, even if 
associated with amaurosis, there is response of the pupil to light im- 
pulse, while in another class of cases there is failure of the iris to 
contract under the influence of light. Again, the dilatation of the pupil 
may or may not be associated with paralysis of accommodation. In 
many of the cases of mydriasis there is amaurosis, and the mydriasis 
disappears when sight is restored, but Aurand and Erenkel think that it 
may occur quite independently of amblyopia, or even a greater deprecia- 
tion of vision. Thus, Parinaud and Richer report a case of hysteria 
with paralysis of accommodation and mydriasis, Harlan one of mydria- 
sis, accommodation-paralysis, and blepharospasm, while, according to 
De Wecker, in hysteria the mydriasis may be associated with cyclo- 
plegia or exist without it. Touching the question of whether the 
mydriasis is a paralytic one or not, Schwarz maintains that although it 
is often so stated, such statement is not necessarily based upon accurate 

52 Revue de Medecine, xvi. 1896. 

53 Festschrift zur Feier des aehtzigjahrigen Stiftungsfestes des Aerztlichen Ver- 
eins zu Hamburg, Leipzig, 1896. 

54 Loc. cit., p. 628. 

65 Zeitschr. f. Augenheilk. 



648 THE EYE AND NERVOUS SYSTEM 

observation. Even when the pupil, widely dilated, does not react to 
light, it is not necessary that a paralytic mydriasis should be assumed. 
A pure paralysis of the iris movement not brought into existence by a 
medicament can be stated to exist only if the pupil promptly responds to 
the action of pilocarpin or eserin. Even the presence of hysterical 
cycloplegia does not prove, according to him, that the mydriasis is 
paralytic, for, on the one hand, one may have paralysis of accommodation 
without pupil paralysis, as, for example, in diphtheria, and, on the 
other hand, as has already been shown, the so-called hysterical accommo- 
dation-paralysis is not a paralysis in the true sense of the word. The 
cases of paralytic mydriasis reported by Boeder, Donoth, and Nonne and 
Beselin have not been accepted by all authors. It has been suggested, 
for example, that atropin might have been used without knowledge, and 
although in Nonne and Beselin's case eserin failed to contract the pupil, 
and a central paralysis and a spastic mydriasis were undoubtedly ex- 
cluded, and although the patient was in a certain sense watched in so 
far as the use of atropin is concerned, Schwarz is evidently unwilling to 
accept the paralytic nature of the mydriasis, which these authors, how- 
ever, do not hesitate to maintain, describing it as an undoubted paresis of 
the sphincter of the iris in a case of typical grand hysteria. They 
record a second case which they describe as hysterical ophthalmoplegia 
interna. Liebrecht, on the other hand, remarks that he has never 
seen an undoubted case of hysterical internal ophthalmoplegia. 

As has already been stated, mydriasis in the absence of paralysis of 
accommodation is due to a spasm of the dilator muscle. To explain the 
paralytic mydriasis, Nonne and Beselin, as well as Donoth, assume an 
abeyance of the cortical centre governing the functions of the sphincter 
of the iris and the muscle of accommodation, and in this respect estab- 
lish an analogy between the condition described in so far as etiological 
considerations are concerned, and hysterical amblyopia, amaurosis, dys- 
chromatopsia, and concentric visual field contraction. Binswanger, 
although he does not doubt the occurrence of pupillary rigidity or 
pupillary sluggishness as an hysterical symptom, warns against its being 
mistaken for reflex light rigidity in the narrow sense of the term, which 
belongs to the domain of the organic diseases of the brain and spinal 
cord, pupillary rigidity in hysteria being associated with myosis and 
spasm of accommodation and convergence. Deutschmann, Wilbrand, 
and other observers also point out the necessity of making a distinction 
between a true reflex pupillary rigidity and mydriatic pupils which do 
not react either to light or accommodation. In response to such objec- 
tion Nonne and Beselin point out that they do not describe reflex pupil- 



XEUROSES AXD PSYCHOSES 649 

lary rigidity, but only mydriatic pupils which fail to respond to light 
and convergence, and again maintain that the condition is due to func- 
tional injury of the cortical centres for the innervation of the sphincter 
or of the dilator. 56 

Pupil dilatation may exist for a short time, for a comparatively long 
period, and in rare instances, — for example, in Harlan's and in Xonne 
and Beselin's cases, — for several months or even years. The pupil 
may be made to contract by suggestion, hypnotism, and by causing the 
patient, as in Pichler's case, to read through a plane glass. 

Thus far we have spoken particularly of bilateral dilatation of the 
pupil. There may be, however, a unilateral mydriasis, sometimes asso- 
ciated with paresis of accommodation and sometimes without such 
association, and there may be dilatation of the pupil with preserved but 
sluggish reaction to light and so-called hysterical paralysis of accommo- 
dation. Gilles de la Tourette describes a hypothetical unilateral mydria- 
sis in total amaurosis of one or both eyes as the result of the loss of the 
light reflexes, which under normal conditions influence the pupillary 
reaction, but Binswanger, commenting on this case, thinks that there 
are some cases in literature belonging to this category, — for example, 
those reported by Mendel. H. A. Hare and myself have recorded 57 
complete amaurosis with mydriasis of the right side, the dilated pupil 
reacting sluggishly to light impulses and moderately to eserin, with 
marked myosis of the left side and moderate amblyopia, in a hysterical 
woman, which had existed for many years, the use of drugs being 
absolutely excluded by keeping her under the observation of trained 
nurses for weeks at a time in a hospital room. 

In addition to immobility of the pupil there is sometimes an inter- 
change of myosis and mydriasis, and Lagovides reports a case of hys- 
terical alternating and intermittent mydriasis, while Blok has observed a 
case of mydriasis and accommodation-paralysis at first alternating from 
the right to the left side, but later stationary in both eyes, associated 
with strabismus convergens. 

Before dismissing this subject the condition of the pupils during 
hysterical attacks remains to be described. We have already noted that 
pupil immobility may occur during an attack, at the end of an attack, or 
without evident association with an attack, although permanent pupil 
immobility has not been observed. 

Striimpel, Oppenheim, and Mobius at one time maintained that a 

56 For further discussion of this subject and the views of neurologists and oph- 
thalmologists, see Binswanger, loc. cit., p. 637. 
"American Medicine, April 21. 1901. 



650 THE EYE AND NERVOUS SYSTEM 

differential diagnosis between epilepsy and hysteria could be made by 
observing the pupils, which in the former were wide and reactionless, 
while in the latter affection their reaction was preserved. Karplus 58 
first, in 1896, observed pupil-immobility during hysterical attacks, and a 
year later Westphal established the accuracy of Karplus' s contention 
that the state of the pupils was not sufficient to make a differential 
diagnosis between epilepsy and hysteria. Karplus still later accurately 
investigated a series of cases of spontaneous and induced hysterical 
attacks, during which the pupils were dilated and immobile. He con- 
siders reflex pupil immobility a frequent phenomenon in paroxysmal 
hysteria, and believes that it depends upon a cortical origin, the pupil- 
dilatation and pupil-contraction being represented in the cortex, and 
that a tonic cramp of the muscular structure of the iris can occur, and 
therefore light reaction not take place, exactly as a tonic cramp of the 
legs in hysteria may be responsible for the elimination of the patellar 
reflex. 

Finally, we may have in hysteria a paradoxic pupil reaction which 
has been reported by Westphal and by Lepine. The last-named author's 
patient showed dilatation of the pupil when light fell upon the exposed 
eyes, this dilatation remaining for a few seconds. The transitory and 
inconsistent changes in the pupil, to use Binswanger's words, which are 
seen in so many hysterical patients, should be regarded chiefly as an 
expression of a pathological perversion by virtue of which psychic 
processes are more easily transmitted to the movement of the pupil than 
under normal conditions. Thus it is that we sometimes have a wide but 
quickly reacting pupil, — that is, one which dilates rapidly under the 
influence of shock, excitement, exertion, etc., — and also, as has often been 
observed, the increase of the natural hippus of the pupil. I have seen in 
one hysterical patient of Dr. Weir Mitchell the most marked example of 
exaggerated hippus that has come under my observation, except as I 
have seen it in one or two cases of insanity. Yon Ketley 59 describes 
a myasthenic action of the pupils in hysteria. (Compare Chapter VI.) 

VI. ANOMALIES OF THE EYELID AND EYE-MUSCLE MOVE- 
MENTS, OR HYSTERICAL SPASMS, CONTRACTURES, PSEUDO- 
PALSIES, AND PALSIES OF THE PALPEBRAL AND BULBAR 
MUSCULATURE. 

1. Blepharospasm. — This condition appears in a clonic, tonic, and 
so-called pseudoparalytic form. 

58 Lehrbuch f. Psychiatrie, xvii. 1898, pp. 1-53. 
69 Wien. med. Wochenschr., Nr. 33, 1901, p. 1559. 



LEUKOSES AND PSYCHOSES 651 

(a) Clonic blepharospasm, common enough in hysteria, is not neces- 
sarily an ocular stigma of this affection. It is characterized by a vibra- 
tory and continual blinking of the lids during the attack. Usually 
bilateral, clonic spasm of one orbicularis appearing in the form of 
fibrillary contraction has been observed. Frequently part of the symp- 
tomatology of an hysterical seizure, it may arise independently of such 
association. In one form of the affection described by Bernutz as the 
attenuated variety, there is a constant vibration or trembling of the 
closed or half-closed lids, which does not cease even during sleep. 

(b) Tonic Blepharospasm. — This is characterized by a convulsive 
closure of the eye, and, unlike the clonic variety of this affection, it is 
frequently, if not usually, unilateral, or at least is more marked upon 
one side than the other, and the spasm may involve the neighboring 
muscles of the face and neck. The skin of the lid is forcibly folded 
and the palpebral borders are vigorously applied one against the other. 
If an effort is made to raise the lid, — for example, by means of the 
finger, — a definite resistance is demonstrable ; and if the patient under 
command tries to open the eye, the only response is a trembling move- 
ment without separation, or without distinct separation, of the ciliary 
margins which have been in contact with each other. Along with this 
effort there may be a distortion of the face and contraction of other 
muscles, — for example, those opening the mouth. Quite commonly 
blepharospasm is associated with intense photophobia, convulsive dis- 
placement of the eyes in different directions, dyschromatopsia, spasm of 
accommodation, and amblyopia. Indeed, it was originally believed by 
Hocken that blepharospasm always accompanied amaurosis, although we 
now know that such is not the case. 

Two clinical varieties have been described, the painful and the non- 
painful. The former, usually bilateral, may be accompanied by the 
visual troubles before named, especially lachrymation and photophobia, 
while the latter, or the non-painful form, is frequently unilateral. 
Gilles de la Tourette has described in painless blepharospasm an anes- 
thetic zone around the eye extending for a distance of two or more 
centimetres above and below the orbit. The conjunctiva is insensitive, 
although the cornea may still retain its sensibility, except at the internal 
portion. In painful blepharospasm with photophobia the zone of anes- 
thesia is replaced by one of hyperesthesia, which may extend to the 
cornea. Richer and Gilles de la Tourette have noted that compression of 
the supraorbital region may cause momentary disappearance of the 
blepharospasm, while pressure upon other areas in the neighborhood of 
the eye may cause an exacerbation or a relapse after quietude. Almost 



652 THE EYE AKD NERVOUS SYSTEM 

complete cessation of the blepharospasm of one of my patients could be 
induced by hard pressure in the neighborhood of the supraorbital 
foramen. Furthermore, the blepharospasm — as, for example, in Ze- 
hender's patient — may be exaggerated or called into existence by press- 
ure on the first cervical vertebra or on the sternal region or, as in Seelig- 
miiller's case, on the lower jaw in the neighborhood of the molar teeth. 

The condition may develop without any external cause in conjunc- 
tion with or after an hysterical crisis, or very frequently in the ordinary 
reflex manner, after a conjunctivitis, a corneal affection, or a sudden 
flash of light. Sometimes the blepharospasm arises suddenly and dis- 
appears with suddenness or, the blepharospasm being complete, con- 
vulsive movements of the lids in the form of exacerbations occur spon- 
taneously, lasting from a few minutes to several hours. Contracture of 
the orbicularis does not always disappear suddenly; and Charcot and 
Gilles de la Tourette have published cases in which it has lasted for 
several years. Again, as in Harlan's patient, there may be an inter- 
mittent violent blepharospasm of one eye, with contraction of the pupil, 
spasm of accommodation, and narrowing of the visual field. 

Binswanger regards blepharospasm as a concomitant of the general 
pathological tendency in hysteria to the formation of contractures. 
According to Gilles de la Tourette, its origin should be considered 
central. Other authors believe that contraction of the orbicularis, at 
least certain varieties of it, are due to hyperesthesia of the retina, which 
causes a reflex closing of the lid to protect the eye from the excessive 
light. 

(c) Pseudoparalytic Ptosis. — In spite of this name, which we owe to 
Parinaud, as we do also in large measure the differentiation of the 
affection from a true ptosis, this condition is not the result of levator 
weakness, but depends upon a cramp or spasm of the palpebral portion 
of the orbicularis. Therefore it would seem that the other term spastic 
ptosis is the more appropriate one. 

According to Parinaud, pseudoparalytic ptosis resembles palsy of 
the levator because the upper lid droops without wrinkling the skin of 
the lid, and if the patient attempts to open the eye there is a con- 
traction of the occipitofrontalis similar to that which one sees in real 
paralytic ptosis. The lid raised with the finger, however, when released 
falls more rapidly than in paralytic ptosis, and the patient may, if his 
attention is directed elsewhere, voluntarily elevate the lid, or as dusk 
comes on, because photophobia and amblyopia are frequently accompany- 
ing symptoms, perform the same act. According to Charcot and Lan- 
dolt, in true paralysis of the levator the eyebrow is elevated, inasmuch as 



NEUROSES AND PSYCHOSES 



653 



the patient endeavors to lift the eyelid by contracting the occipito- 
frontalis, while in pseudoptosis, which is really due to a partial spasm 
of the orbicularis, the eyebrow follows the contraction of this muscle and 
descends below the normal level, and there is obliteration of the wrinkles 
on the corresponding side of the forehead (Fig. 10). This important 
observation is of particular interest because we may have, as Charcot 
has shown, a simulation by hysteria of the syndrome of Weber, — that is 




Fig. 10.— Hysterical spastic ptosis. (After Wilbrand and Saenger.) 



to say, a ptosis on the side opposite to hemiplegia. It would seem, how- 
ever, that this so-called eyebrow-sign is not a constant one, as Wilbrand 
and Saenger have shown, and that the other indications referable to 
blepharospasm must be sought out and examined. Sometimes the 
depressed eyebrow is straighter than the one on the opposite side; the 
overhanging folds of skin of the upper lid are converted into a series of 
parallel or concentric folds, the free border of the lid is curved, the 
occipitofrontalis muscle comparatively smooth, and the palpebral fissure 
not entirely closed. Finally, there may be some resistance to the finger in 
the effort to raise the lid, and the pseudoptosis does not disappear when 
there is over-extension of the head. Binswanger, who elaborates these 
differential diagnostic points, thinks they are important to support the 
diagnosis of pseudoptosis in doubtful cases, and calls attention to the 
fact that a sharp distinction between spastic ptosis and fully developed 
blepharospasm is often difficult, especially as there are transitory forms 



654 THE EYE AND NEKVOUS SYSTEM 

■ — as, for example, in Zehender's case, in which blepharospasm termi- 
nated in pseudoptosis. 

Although spastic ptosis is usually unilateral, bilateral varieties of 
the affection have been reported, and the condition may be accompanied 
by hemispasms elsewhere, — for example, glossolabial hemispasm, as in a 
case of Gilles de la Tourette's. Binswanger points out that hyperes- 
thesia of the retina may be a precursor of pseudoptosis, and, quoting 
Bardol's case, insists that sometimes psychic causes alone are operative. 
He agrees with Wilbrand and Saenger that a palpebral spasm brought 
about, as in the case recorded, by a flash of lightning, represents a patho- 
logical persistence of the voluntary closing of the lids to shut off the sight 
of the storm, and demands that spastic ptosis in hysteropathic individ- 
uals with overexcitability in the sensory sphere should be regarded as a 
pathological exaggeration of physiological, cortical, and intracortical 
reflex. 

2. Hysterical Paralysis of the Orbicularis. — According to Gilles 
de la Tourette, the title just given is a misnomer, a's palsy of the orbicu- 
laris has not been proved to occur in hysteria, and Wilbrand and Saenger 
are not able to report a hysterical paralysis of this nature. It is true 
that Gilles de la Tourette refers to transitory lagophthalmos during hys- 
terical attacks, forming a part of a group, as he expresses it, of con- 
tractures and spasms difficult to analyze. Even in so-called hysterical 
facial paralysis, the orbicularis seems to be respected. 60 

3. Hysterical Spasm of the Levator. — According to Koenigshofer, 61 
hysterical cramp of the elevator of the upper lid has, up to the present 
time, been observed only once. No reference is given, but doubtless the 
statement is based upon the case which has been reported by Gold- 
scheider, and which is summarized by Wilbrand and Saenger. 62 The 
patient, in addition to cramp of the levator, was affected with a cramp- 
like contraction of the inferior rectus, which appeared when the gaze 
was directed below and to the right. Parinaud has observed the simu- 
lation of Graefe's sign in exophthalmic goitre by contracture of the 
levator ; intermittent retraction of the lids appeared even when the eyes 
were at rest. Garl Kunn 63 has described cramp of the levator which 
caused decided elevation of the upper lid and widening of the palpebral 
fissure. 



60 For further discussion of this subject, see Gilles de la Tourette, Traite" de 
l'Hysterie, I. partie, 1891, p. 415. 

61 Encyklopiidie der Augenheilk., Lieferung 10, Leipzig, 1904, p. 417. 
02 Die Neurologie des Auges, Bd. L, Wiesbaden, 1900, p. 71. 

68 Deutschmann's Beitrage zur Augenheilkunde, iii. 1898. 



NEUROSES AND PSYCHOSES 



655 



4. Hysterical Paralysis of the Levator, or Flaccid Hysterical 
Ptosis. — As is well known, the existence of hysterical ptosis in the 
form of a flaccid relaxation of the levator was denied by Charcot and is 
doubted by Borel and Gilles de la Tourette. Their contention is that 
hysterical ptosis, as already noted, is due to spasm of the orbicularis 
and not to paralysis of the levator. On the other hand, equally capable 
observers, notably Schmidt-Eimpler, Kempner, Hitzig, Kieman, and 
Wilbrand, 64 have reported flaccid ptosis in hysterical subjects corre- 
sponding completely to the clinical picture of true paralysis of the 
levator (Fig. 11). Abadie has reported paralytic ptosis of intermittent 
form and of hysterical origin. 




Fig. 11.— Flaccid hysterical ptosis. (After Wilbrand and Saenger.) 

Undoubtedly flaccid ptosis, as compared with pseudoptosis, is rare. 
Usually bilateral, it has also been observed by Schmidt-Rimpler, Wil- 
brand, and myself as a unilateral affection. It may appear suddenly or 
develop gradually, and is usually seen in those individuals who exhibit 
other well-marked hysterical phenomena, — for example, contractures of 
the bulbar eye muscles, amblyopia, and amaurosis, visual field restric- 



64 For a discussion of the literature of this subject, see Wilbrand. Archiv f. Augen- 
heilk., xxxix. 1899, p. 172, and Wilbrand and Saenger, Die Neurologie des Auges, 
Bd. i. pp. 463-477. 



656 THE EYE AND NERVOUS SYSTEM 

tion, and spasm of accommodation. It may be caused by local irritation 
or be one of the symptoms of an attack. 

Schmidt-Rimpler regards the condition as a simple, often voluntary, 
relaxation of the levator, exactly as it occurs in sleep, while Hitzig 
ascribes it to an actual paralysis. If the development has been grad- 
ual an autosuggestive origin may be ascertained. 

It is conceivable that simulation, even of unilateral flaccid ptosis, is 
possible, although on this point Wilbrand and Saenger have expressed 
serious doubts. 

A differential diagnosis between flaccid hysterical ptosis and some of 
the forms of pseudoptosis is often difficult, and those interested in the 
literature and comparison of the cases should consult Wilbrand and 
Saenger. 65 The similarity of hysterical ptosis and the ptosis of organic 
disease, — for example, that of ataxia, multiple sclerosis, bulbar palsy, 
and even meningitis, — is often striking. To distinguish them Landolt 
proceeds as follows: The subject of ptosis is seated in front of the 
observer and commanded sharply to look at one of his eyes. To accom- 
plish this the patient inclines the head strongly backward, so that the 
gaze may pass under the drooping lids. The observer now puts his hand 
on the top of the patient's head, who continues to regard the same fixa- 
tion point, and insensibly raises and even inclines it gradually forward. 
If the pupils of the subject remain uncovered during this operation, 
hysteria may be suspected, because to effect this the lids would have to 
raise themselves with the eyes while the face is lowered. In other 
words, as Landolt expresses it, to keep the eyes open, whilst the head is 
inclined forward is equivalent to raising the eyes and lids when the 
head is straight, a thing absolutely impossible in paralysis of the 
levator, in which the patient ceases to see the object looked at as soon as 
the head leaves the compensating position, because the lids immediately 
cover the pupil. 

Whether the so-called morning ptosis of Gowers, the night ptosis of 
Weir Mitchell, should be classed as a variety of flaccid ptosis is some- 
what doubtful. This phenomenon will be referred to again in the 
section on neurasthenia, and may be dismissed now with the statement 
that certain nervous women, particularly on awakening in the morn- 
ing, may for some minutes find it impossible to raise their eyelids with- 
out assisting the action of the levator with their fingers. 

The hysterical anomalies of the external eye muscles — that is, of the 
bulbus musculature — next claim attention. 



66 Loc. cit., pp. 463-477. 



LEUKOSES AND PSYCHOSES 657 



I.— ANOMALIES OF CONVERGENCE. 

Cq\ Convergence-Insufficiency and Convergence-Paralysis. — The asso- 
ciation in hysteria of insufficiency of convergence and weakness of the 
amplitude of accommodation has been referred to. Owing to the inti- 
mate relation existing between accommodation and convergence, if the 
patient makes but feeble efforts of accommodation, or, more accurately, 
is unable to make but feeble efforts of accommodation when looking 
at a near-point, a tendency to diminution of the convergence-action will 
appear, resulting in convergence-insufficiency, or, as it is sometimes 
called, a relative insufficiency of the interni. Ordinarily, under nor- 
mal conditions, the amplitude of convergence is equivalent to the differ- 
ence between the maximum and the minimum of convergence, or, stating 
the proposition in metre angles, the normal average of maximum con- 
vergence is 9.5 metre angles and the average minimum of convergence 
is — 1 metre angle, the amplitude of convergence being, therefore, 10.5 
metre angles. In hysteria this amplitude of convergence is frequently 
considerably reduced at both extremities. In other words, the far-point 
of convergence is brought nearer to the patient, while the near-point is 
farther removed from him, the anomaly, as Parinaud has shown, pre- 
senting the same characteristics as those which pertain to hysterical 
disorders of accommodation, as described on page 29. In order to 
determine a condition of this kind, the ordinary tests with prisms are 
required, diplopia not being spontaneously present. Exophoria for 
distance may be slight or absent, but is present at the near-point. 
The convergence near-point usually is less than three inches from the 
nose and may be removed to many inches from that point. Some- 
times the patient is able to converge on an object, — for example, a 
pencil-point, — when brought close to the eyes, in excessive degree, but 
can maintain this convergence only momentarily. 

The convergence-insufficiency may be so great that it constitutes a 
convergence-paralysis, & condition which has been observed in hysteria 
by Parinaud, Borel, and Schweigger. Although either eye can be 
moved inward to a normal degree when the other eye moves outward, its 
internal rectus does not respond to the impulse of convergence, and the 
convergence near-point, in place of being a few inches from the eyes, is 
removed to a point one or two metres from the eyes, and if the test- 
object is advanced within this point, insuperable crossed diplopia at 
once becomes manifest. A convergence-insufficiency depending upon 
structural or insertional anomalies of the interni, which has resulted in 
the development of a hysterical convergence-paralysis, may by operative 

42 



658 THE EYE AND NERVOUS SYSTEM 

interference, — for example, a tenotomy of the externus, — be followed, as 
Schwarz and Borel have shown, by sharp convergence-excess or spasm, 
which in its turn may be cured by suggestion. 

(b) Convergence-Excess and Divergence-Paralysis.. — An abnormal tend- 
ing inward of the visual lines may depend upon excessive convergence, or 
deficient divergence, or upon these conditions combined. 66 

Just as a convergence-insufficiency may be replaced by an actual 
convergence-paralysis, so also a divergence-insufficiency may be replaced, 
as Parinaud, UhthofT, Straub, von Hippel, and many other observers 
have noticed, by an actual divergence-paralysis. This condition mani- 
fests itself by homonymous diplopia and convergent strabismus when the 
eyes are fixed upon a distant point. As the test-object approaches the 
patient, and especially on lateral fixation, there is diminution of the 
convergent strabismus and of the diplopia, and finally a point is reached 
at one or two metres from the eye at which single vision and orthophoria 
are developed, but within this limit there is exophoria and crossed 
diplopia. Now this diplopia is peculiar in that, no matter whether it is 
homonymous on the distal side of the point at which single vision is 
evident, or whether it is crossed on the proximal side of this point, the 
images do not separate to any perceptible degree when the test-object is 
moved in the lateral direction from side to side. According to Pari- 
naud, in some cases, although it is possible to obtain fusion at the point 
already indicated, in others such fusion is impossible, and the crossed 
diplopia suddenly becomes homonymous diplopia. There is, to use his 
phraseology, a horror of binocular single vision. This hysterical di- 
vergence-paralysis is due to spasm of convergence and not to paresis of 
divergence. It depends, as Kunn remarks, on cramp of the interni with 
atypical diplopia. 

It therefore becomes evident that in hysterical patients all of the 
factors connected with convergence may be defective, accommodation, 
fusion, and probably also " the sense of convergence" (Parinaud, Han- 
sen Grut), and we may have an absolute or a relative weakness of the 
adductors or a disturbance of central origin, which creates a weakness 
or a total disappearance of the power of fusion. 

We have already seen, on page 27, that painful, hysterical visual 
sensations are largely connected with the anomalies of accommodation. 
Necessarily, however, as these anomalies are associated with those of 
convergence just detailed, the combination produces the highest type of 
so-called hysterical asthenopia, and while it may be true that the defects 

60 For the tests to determine these conditions, see chap. v. 



NEUROSES AND PSYCHOSES 659 

of accommodation are the predominant ones, as a rule both convergence 
and accommodation are involved, and there arise photophobia, retinal 
anaesthesia, obscuration of vision, headache, inability to maintain fixa- 
tion, radiating pain, not only in the head but extending to the neck, 
shoulders, and chest, all aggravated by an attempt to use the eyes, but 
often present without ocular efforts at close ranges. 

Kcenigshofer 67 thinks that in one class of cases a characteristic con- 
dition is that the patients cannot fix any one object, no matter at what 
distance from it they are. As soon as the attempt is made, all objects 
swim before their eyes, and vertigo and nausea are predominant signs. 
These patients direct their visual axes and accommodation upon a 
definite distance, so that fixation upon a nearer or further point is not 
possible, or possible only to a limited degree. For this form of muscular 
asthenopia he wishes to suggest the term " convergence immobility" 
( Convergenzstarre ) . 

(c) Convergence-cramp or Contracture, and Hysterical Convergent Stra- 
bismus. — We have seen in the preceding paragraphs that in hysteria 
there may be convergence-excess, although it should not necessarily be 
regarded as an essential sign of the disease. What may be characterized 
as an advance on convergence-excess receives the name of convergence- 
cramp, or spasm, which certainly is a frequent hysterical phenomenon, 
and when well developed manifests itself by inward — that is, con- 
vergent — strabismus and is usually associated with spasm of accommo- 
dation. 

The idea of spastic strabismus as part of the symptomatology of 
hysteria dates to the observation of Landouzy, who remarks that in the 
course of hysterical attacks we may notice a deviation of the eyes, to 
which some pathologists have applied the term hysterical strabismus. 
The earlier literature of this subject has been analyzed by Gilles de la 
Tourette, 68 to whose treatise those interested should turn. In more 
recent times detailed description of spastic hysterical strabismus will be 
found in the work of Borel, 69 and particularly good reports of this con- 
dition have been recorded by Manz, Schweigger, Ulrich, and Xonne and 
Beselin. 

We have already seen that contracture of convergence, or so-called 
spastic strabismus, not infrequently accompanies blepharospasm, and 
have also noted that a condition of this character may be seen in asso- 



Ophthalmologische Klinik, 1902, vi. p. 113. 

Loc. cit. 

Archives d'Ophthalmologie, 188G-1887, and Annales d'Oculistique, 1897-1898, 



660 THE EYE A1STD NEKVOUS SYSTEM 

ciation with hysterical ptosis, — for example, a rotation of the eyeballs 
downward and inward, as in Hitzig's case, followed later by convergent 
strabismus ; inward and upward, as in a case reported by Wilbrand and 
Saenger; or into a position of permanent convergence, which was, 
indeed, never entirely overcome, as recorded by Oppenheim. 

Convergence-spasm and spastic strabismus, unaccompanied by anom- 
alies of lid movement, in the form of blepharospasm or ptosis, occur 
and are characterized by convergent squint, limitation of the motility of 
the external recti, and by homonymous diplopia. The condition resem- 
bles paralysis of the abducens, for which it may be, indeed has been, 
mistaken. But, under these circumstances, double images do not be- 
have as they would in a paralytic case. They have, as Ulrich expresses 
it, the same separation on looking to the right as when the patient looks 
to the left, a peculiarity which was exceedingly marked in a case re- 
cently under my own care, in which, with every attempt at fixation, 
there was excessive convergence and marked limitation of the external 
recti movements, but no alteration in the apparent distance between the 
double images, no matter to what portion of the lateral field of fixation 
they were moved. Schwarz insists that strict attention should be paid 
to the secondary deviation. If this is not greater than the primary de- 
viation of the squinting eye, the condition is probably not one of sixth- 
nerve palsy, especially as one is usually able to establish in an eye of 
this character spastic myosis, spasm of accommodation, micropsia, and 
sometimes monocular diplopia. Sometimes, however, the diagnosis is 
extremely difficult, as Schwarz himself admits, as is evident from an 
examination of the case reported by Nonne and Beselin. Schwarz 
believes that the only positive differential diagnostic point between a 
convergence-cramp and an abducens paralysis is developed by an ob- 
jective investigation of the optical apparatus. In a case of his own he 
was able to determine by a skiascopic examination that in one direction 
of the gaze at which the double images were evident an accommodation 
of more than 2 D. developed. JSTonne and Beselin point out that in 
spasm, in contradistinction to palsy, contractions of the muscles inner- 
vated by the facial, dread of light and pain in and around the affected 
eye would be apt to be present. 

Hysterical spastic strabismus, or, more accurately, as Parinaud 
properly insists, contracture of convergence, may occur as the prodrome 
of an attack of grave hysteria, during hysterical attacks and at their 
termination, but is, in general terms, a transitory phenomenon, although 
it may terminate in some form of deviation, — for example, a conjugate 
deviation, — exactly as a conjugate deviation may sometimes precede a 



NEUROSES AND PSYCHOSES 661 

convergent strabismus. Such contracture of convergence, or strabismus, 
may arise in association with other muscular movements — as, for ex- 
ample, in Shuster's patient with traumatic hysteria, in whom when the 
arm was raised strabismus developed. It can be brought about, as other 
hysterical phenomena, by suggestion and cured in like manner. Perma- 
nent convergent strabismus in hysteria must not be regarded as a stigma 
of the disease. An investigation will always show other factors upon 
which this depends, notably hyperopia, anisometropia, amblyopia of one 
eye, and absence of the fusion sense. In a notable case under my own 
observation of long-standing major hysteria, to which reference will 
again be made, a prolonged conjugate deviation of the eyes to the left 
lasting for many months was followed upon recovering by well-marked 
convergent strabismus, occurring, as is usually the case in hysteria, in 
crises, but finally settling to a well-marked moderate inward strabismus. 
When investigation was thoroughly made a high degree of hyperopia, 
with all of the symptoms of convergence-excess, was found, and suit- 
able glasses produced parallelism of the visual axes. 

While it is true that in the convergent strabismus of the character 
which has been described as hysterical there may be limitation of the 
outward movements of the eyes, it should be remembered that this is 
not always the case, and that one sign in the minor degrees of con- 
vergence-contracture which helps to differentiate it from an abducens 
palsy is that the field of fixation is normal in the one and naturally 
limited in the other. When the convergence contracture is excessive, 
then this test cannot be applied, although it does not in the least destroy 
the very evident etiology of the affection as an anomaly of convergence 
innervation. 

Spasmodic Conjugate Deviation. — In discussing spastic strabismus in 
hysteria, we have seen that there may be a spasm or cramp of an indi- 
vidual muscle, or in association with blepharospasm, or various types of 
ptosis, and have noted in detail the well-marked hysterical phenomena 
of convergence-cramp and a strong inward strabismus, often combined 
with spasm of accommodation. We have now to deal with varieties of 
conjugate deviation, in which a spasmodic condition of associated muscle 
groups is evident. During clonic or tonic hysterical convulsions there 
may be marked contraction of the ocular muscles and with this a coin- 
cident contraction of the muscles of the neck, giving the appearance 
of a conjugate deviation of the head and eyes, or, again, under similar 
conditions, the eyes may be turned in any direction by what Pansier 
calls convulsive rollings, without any apparent rule guiding the irregu- 
larity of the spastic rotations. These are not true forms of spastic 



662 THE EYE AND NEKVOUS SYSTEM 

strabismus. Both eyes execute incessant movements but are not per- 
manently deviated. They seem, to use the language of Parinaud, to 
have lost the power of fixation. Often these cases may be complicated 
with photophobia and blepharospasm, and there may be lagophthalmos 
owing to a contracture of the levator palpebral as pointed out by Levy 
and described by Gilles de la Tourette and Parinaud. Such irregular 
contractures of the ocular muscles may be accompanied by diplopia, or, 
more accurately, by attacks of diplopia, occurring sometimes frequently 
during a single day. 

In typical spasmodic conjugate deviation the contractures affect the 
associated parallel movements, or, as Parinaud puts it, the associated 
movements of direction, and while it is true that the deviations may be 
in any direction, according to the same author, the inferior and oblique 
position is preferred. As may have been inferred, the condition is not 
commonly a permanent one, occurring as it does during seizures, and 
yet what may be designated permanent deviations of well-marked char- 
acter have been recorded. Notable in this respect is the case of Erost, 70 
in which the eyes were directed downward and to the right, upward 
movements being almost impossible, and the condition lasted one year. 
I studied with Dr. Wharton Sinkler, of this city, a case of major hys- 
teria in which there was pronounced unvarying conjugate deviation 
upward and to the left, with lagophthalmos, owing to contracture of the 
levator palpebral of each eye, which lasted for nine months. In less 
persistent cases there may be attacks, lasting for days or weeks, coming 
on with other convulsive seizures; or the type may change — that is, a 
conjugate deviation in one attack may become a spasm of convergence in 
another, as I myself have noted in the case already referred to, and as 
has also been reported by Manz. The combination of contractures of con- 
vergence with loss of lateral movement has already been described, and 
typical cases of this character are not uncommonly seen. 

Ordinarily the contractures upon which depend these conjugate 
deviations necessarily indicate the implication of both eyes simulta- 
neously, but it should be remembered that in unilateral blepharospasm 
there may be, when the patient attempts to fix the affected eye, a prompt 
spasmodic deviation upward and inward. This unilateral condition of 
spasm is illustrated in Goldscheider's case, whose patient exhibited 
nothing peculiar in the rotations of the ocular muscles until she was 
required to look to the right and below, when the left bulbus was spas- 
modically drawn downward and inward, while the left upper lid was 

70 British Medical Journal, 1884. 



NEUROSES AND PSYCHOSES 663 

elevated. These spasms of the ocular muscles, to which Borel makes 
full reference, have already been discussed under spastic strabismus. 
As in other hysterical ocular phenomena, with, these conditions may be 
present amblyopia, photophobia, and blepharospasm, and they may be 
brought into existence by emotion, trauma, intoxication, or reflexly by 
dental irritation, and would seem to be more often found in young 
hysterics, who, curiously enough, apart from their convulsive seizures, 
may have little marked other stigmata of hysteria. 

Hysterical Palsies of the Ocular Muscles : Hysterical Ophthalmo- 
plegia. — (a) Palsies of Individual Muscles. — Binswanger, 71 referring to 
this much disputed subject, gives a summary of what he calls the ap- 
proximately authentic cases of hysterical paralysis of the eye muscles, 
as follows: Paralysis of the left internal rectus and the superior recti 
muscles on both sides (Guttmann 72 ) ; paralysis of the right external 
rectus, later of the left (Duchenne 73 ) ; bilateral paralysis of the ab- 
ducens (Leber 74 ) ; paralysis of the abducens (Parinaud 75 ) ; paralysis 
of the right and later of the left abducens combined with contracture of 
the internal rectus (Borel 76 ); ptosis, paralysis of accommodation, 
paralysis of the internal rectus and of the inferior rectus of the right 
eye (Parinaud and Richer 77 ); paralysis of the abducens, two cases 
(Wilbrand 78 ) ; paralysis of the left inferior oblique (Wilbrand and 
Saenger, examined later by Nonne and Beselin 79 ). In the last named 
case paralysis of the oblique on the left side was made to disappear by 
suggestion, and so-called hysterical paralysis of the right superior 
oblique was later observed. Other cases, not quoted in this summary, 
are these : Ptosis, divergent strabismus, and nystagmus, especially occur- 
ring in children (Steiner 80 ) ; palsy of all eye muscles except the 
internal recti ( Stephenson 81 ) ; double oculomotor palsy of hystero- 
traumatic origin (Debove 82 ) ; ptosis with complete fixation of the left 
eyeball (Cowan 83 ). Schafer's assertion that oculomotor palsy is fre- 

71 Loc. cit., p. 389. 

12 Berlin, klin. Wochenschr., 1869. 

73 Gaz. des Hop., 1875. 

74 Graefe u. Saemische, Handbuch der gesammten Augenheilk., I. Aufl., v. p. 985. 

75 Soc. d'Ophthal. franchise, 1886. 

76 Arch. d'Opth., 1886. 

77 Binswanger, loc. cit. 

78 Jahrb. der Hamburger Staats-Kranken-Anstalten, 1890. 

79 Binswanger, loc. cit. 

80 Jahresbericht f. Ophthal., xxix. 1898, p. 567. 

81 British Medical Journal, 1902, i. p. 205. 
82 Recueil d'Ophthalmologie, xiii. 1891, p. 124. 

83 New York Eye and Ear Infirmary Reports, January. 1901. 



664 THE EYE AND NERVOUS SYSTEM 

quent in children has not been confirmed, and Binswanger regards it as 
erroneous. It is hardly necessary in an article of this character — indeed, 
space does not permit — to analyze the case histories. Such analyses 
have been well made by Binswanger, Mobius, Parinaud, Kunn, and 
many others, and it would seem to be settled with a fair amount of 
definite accuracy that, as Koenig has said, thus far it does not seem 
that any true case of hysterical eye-muscle palsy has been published. 

Furthermore, as Gilles de la Tourette has expressed it, we may 
suggest to a patient palsy, but cramp or contracture always appears. 
Parinaud, to whom we owe so much for his accurate study of hysterical 
cases, and who has himself reported palsy of ocular muscles in hysterical 
patients, remarks : Paralysis of the third pair and contracture limited 
to one muscle, or to the territory supplied by one nerve, probably do not 
exist in hysteria, but he further goes on to say, as hysteria is a disease 
continually presenting new phases, it is prudent to make a reservation 
that such a condition may have been observed and implies that it may in 
the future be observed. He quotes Samelsohn as having seen one case of 
hysterical paralysis of the sixth pair, but disputes Borel's case of 
abducens paralysis, which he thinks on careful analysis indicates a con- 
dition to be explained by the symptom already so much dwelt upon, 
namely, contracture of convergence. He feels sure that any case of 
well-marked paralysis of the third pair in a hysterical patient — for 
example, one that he himself has reported which he observed for a 
number of years — should give rise to the suspicion of another influence 
and of the coexistence of two separate diseases. 

How, then, are these conditions to be explained ? Binswanger holds 
that the so-called hysterical paralyses of the eye muscles always indi- 
cate a defect of the associated lateral movements, or a motor insuffi- 
ciency of certain muscular movements closely connected with psychical 
processes, and sometimes a combination of paretic and spastic motor 
disturbances peculiar to hysteria. I have never seen, in a very large 
experience in hysterical patients, any isolated paralysis of an eye muscle, 
and believe with Parinaud and other writers that the so-called hysterical 
palsies should be explained by what is sometimes described as an asso- 
ciated ocular paralysis, — that is, although the eyes cannot make certain 
movements in which they are usually associated, the directing power of 
the muscles may be unimpaired when they exercise their function in a 
different direction. In other words, there is a paralysis of movement 
and not of the muscles supplied by a given nerve. We have, then, — 

(6) Palsy of associated parallel movements, or, in other words, the 
Blicklahmung of the Germans. Thus, as, for example, in a case 



LEUKOSES AKD PSYCHOSES 665 

of Claude's, there may be inability of the eyes to move to the left, — 
that is, loss of the directing power of the right internus and of the left 
externus, which are associated antagonists; there is loss of lsevo- 
version, using Duane's phraseology. In the same way there may be 
loss of dextroversion, and so on, of the several different associated 
parallel movements. 

(c) Hysterical ophthalmoplegia, many cases of which are on record, as 
Parinaud has well pointed out, is characterized essentially by the fact 
that the voluntary eye-muscle movements are affected, and the patient 
cannot turn or rotate the eye to any designated point, although the same 
movement can usually with ease be involuntarily accomplished. The 
condition has been compared to the characteristics of mobility of the 
lower extremity in hysterical abasia. For example, at present I am 
studying with Dr. Wharton Sinkler, of this city, a case of pronounced 
hysteria in which the patient cannot raise the eyes in the least above 
the horizontal level when directed to do so, although she is perfectly 
able when her attention is directed momentarily away from the thought 
of upward rotation to make this movement. A similar associated 
paralysis of the superior recti muscles is well described by Rogues and 
Sirol. 84 If the upper lids are raised, the upward rotation of the globe 
above the horizontal plane is impossible, although all other associated 
movements are preserved. The movement of elevation, which cannot be 
excluded when the lids are elevated, can be made when the lids are 
closed. It is not necessary to multiply instances. Even in cases of 
conjugate deviation it is possible — as, for example, it was in Frost's 
case, in which both eyes were directed downward and to the right — to 
make the one eye fix perfectly and follow the movements in all direc- 
tions when the other eye was closed, and the same observation has been 
made by Parinaud in one of his patients. Furthermore, these so-called 
palsies disappear during chloroformization. 

Cases of hysterical ophthalmoplegia have been described — for ex- 
ample, by Ballet, Warner, Bristowe, and others — in hysterical patients 
the subjects of exophthalmic goitre, but even here — as, for example, in 
Ballet's case, although there was complete inability to move the eyes, 
which were normally directed — automatic movements were possible. 
Eaymond and Koenig, referring to complete hysterical ophthalmoplegia 
characterized by complete loss of voluntary movement, decline to regard 
them as true paralyses and insist on a differential diagnostic point 
between this condition and a genuine ophthalmoplegia in the absence of 



Annates d'Oculistique, cxxxv. 1901, p. 360. 



666 THE EYE AKD NEBVOUS SYSTEM 

mydriasis. Nonne and Beselin quote cases of palsy of the external eye 
muscles lasting for several days, accommodation and pupillary func- 
tions being normal. These may be explained by assuming a transitory 
spasm of the external eye muscles, which, according to Gilles de la 
Tourette, are common in hysteria and may produce, as has already 
been pointed out, temporary attacks of diplopia. 

(d) Dissociation of the Eye Movements: — According to Kunn, 85 many 
cases of so-called palsy and cramp of the eye muscles in hysteria are 
really dissociations of the eye movements. This condition is character- 
ized, according to him, not by a lasting and constant limitation of 
movement, but by a loss of movements or rotations that were formerly 
associated, and takes place in both eyes, so that exactly in the atypical 
and changing condition of the rotation disturbances the characteristic 
symptom of the condition is contained. Borel has described in hysterical 
patients, and by ingenious experimentation proved, a loss of the muscu- 
lar sense of the eyes. 

Hysterical Nystagmus.. — Binswanger maintains that a genuine hyster- 
ical nystagmus lasting for some time and occurring independently of 
hysterical paroxysms does not occur. Pansier remarks that just as nys- 
tagmus is frequent in epilepsy it is rare in hysteria. He quotes Bouffi- 
net as attributing to it the following characters : It is mixed, — that is 
to say, it exists as well in a state of fixation as in a state of non-fixation ; 
it is unaccompanied by refractive errors; it is permanent and its in- 
tensity can diminish with the disappearance or the diminution of the 
crisis ; but it does not disappear completely and differs from that seen 
in multiple sclerosis, inasmuch as the latter is exaggerated by the move- 
ments of the ocular globes. As is well known, Charcot insisted that 
nystagmus served as a differential diagnostic symptom between dis- 
seminated sclerosis and hysterical affections, being present in the former 
and absent in the latter. Sabrazes thinks he has proved that nystagmus 
should be regarded as a possible hysterical manifestation because of the 
efficacy of suggestion in one of his cases. This inhibited the nystagmic 
movements, and there were in addition the presence of other hysterical 
phenomena. Oscillation or nystagmic movements of the eye may be 
produced by hypnotic suggestion in hysterical patients. A rapid oscilla- 
tion of the eyeball, either in a horizontal or oblique direction, synchro- 
nous with contractions of the orbicularis when resistance is offered to its 
contraction with the fingers, has been recorded by Stransky and is 
described by him as an associated nystagmus. It occurs, according to 

85 Deutschmann's Beitrage f. Augenheilk., vol. iii. 1898. 



NEUROSES AND PSYCHOSES 667 

this author, in neurotic individuals. Santos Fernandez 86 has reported 
hysterical nystagmus. The rapid and sometimes bizarre movements 
of the eyeballs which occur during hysterical attacks, and to which refer- 
ence has already been made, are well known and cannot be regarded as 
examples of true nystagmus. (Compare Chapters V and IX.) 

VII. PREVIOUSLY UNCLASSIFIED HYSTERICAL 
OCULAR PHENOMENA. 

1. Disturbances of Sensation. — Briquet considered anaesthesia of 
the conjunctiva, especially of the left side, as a condition so constantly 
present in hysteria that it might be regarded as characteristic. Schwarz, 
however, maintains that conjunctival and corneal anaesthesia is not 
peculiar to hysteria, but is seen in pure neurasthenic cases. This is 
contrary to my experience. The entire conjunctival expansion may be 
insensitive, or the anaesthesia may be confined to certain areas, par- 
ticularly to an area below the cornea. The tarsal conjunctiva is less 
frequently anaesthetic than the bulbar mucous membrane. The cornea, 
more uncommonly than the conjunctiva, is involved in anaesthesia. 
According to Gilles de la Tourette, this is usually incomplete, the 
centre of the cornea retaining its sensibility. Fere, as already noted, 
believed that hysterics with hemianaesthesia unassociated with restriction 
of the visual field and achromatopsia did not lose the sensibility of their 
conjunctivas, but if they had dyschromatopsia and concentric contrac- 
tion of the field of vision the conjunctiva would be anaesthetic, while if 
there was achromatopsia and almost complete loss of indirect vision, 
both the conjunctiva and cornea would be insensitive. This observation 
lias not been confirmed by other observers. The semeiological value of 
corneal and conjunctival anaesthesia in hysteria has recently been dis- 
cussed by Robinau. 87 

In moderate grades of conjunctival anaesthesia the palpebral reflex is 
retained, while in higher grades it is lowered or lost. It may be lost, too, 
if the cornea, being anaesthetic, is touched, but if the test-object comes 
into the field of the pupil there is a response. This, however, is not 
due to contact, but is a retinal lid-closure reflex. The lachrymal reflex 
is retained even when the conjunctiva is wholly insensitive, as has been 
shown by Pitres, Gilles de la Tourette, Spiller, and others. This preser- 
vation of the glandular reflex, as Spiller suggests, may serve to dis- 
tinguish hysterical from organic anaesthesia. In place of anaesthesia of 
the conjunctiva and cornea there may be hypera?sthesia of these mem- 

86 Clin. Opht., Paris, 1904, x. 345. 

87 Bordeaux These, 1904. 



668 THE EYE AKD JSTEKVOUS SYSTEM 

branes and often associated with it amblyopia, lachrymation, photo- 
phobia, and orbicularis spasm. 

Anaesthesia of the skin of the lids and surrounding cutaneous sur- 
face is also present in hysterics, but not so frequently as the same con- 
dition of the oculo-mucous membrane. As before noted, Gilles de la 
Tourette has described a periorbital zone of anaesthesia in non-painful 
hysterical blepharospasm, which in the painful variety of the affection is 
replaced by a similar hypersesthetic area. 

Spots sensitive to pressure in the neighborhood of the eyes are 
common in hysteria, especially along the inferior orbital border. These 
tender points Schwarz considers to be quite characteristic of hysteria in 
the absence of neuralgia, and if they do not represent the position 
of a nerve-trunk or one of its points of exit. 

Hysterogenetic zones in and around the eye — for example, in the 
infra- and supraorbital region — have been described, and Lichtwitz has 
recorded a case where the conjunctiva, cornea, and lachrymal canals 
were occupied by hypnogenic, letharogenic, and spasmogenic zones, inter- 
ference with which produced conditions indicated by their descriptive 
names. 

2. Disturbances of Secretion. — Excessive secretion of tears, some- 
times paroxysmal in nature, is frequent in hysterical patients, and is 
sometimes called hysterical epiphora. It is often accompanied by 
spasm of the orbicularis. Bloody tears — i.e., bleeding from the outer 
angles of the eyes — and the so-called vicarious menstruation have been 
described by Hensinger and others in subjects of hysteria (page 342). 

Chromidrosis, which consists of a bluish-black discoloration of the 
lids, somewhat oleaginous in nature, and which can be wiped away, is 
occasionally encountered in hysterical subjects, but is not necessarily a 
stigma of the disease. Hysterical chromidrosis lasting for months has 
been described by Putnam. Severe cases of oily seborrhoea have been 
reported in which the dusky secretion has been allowed to accumulate 
for several years, and in a remarkable example of this nature recorded 
by Van Harlingen 88 a thick black mask of the face, covering the fore- 
head and nose, had been allowed to form. 

3. Chromatopsia. — Strzeminski 89 has reported erythropsia as a 
rare phenomenon in hysteria and refers to similar observations by Hil- 
bert. Eano had a patient who saw in the' field of vision spots of color, 
and Meirowitz 90 one who when looking into the air detected colored 

88 American Journal of the Medical Sciences, cxxvii., No. 2, 1904, p. 245. 

89 Recueil d'Ophthalmologie, 3 Series, 1902, xxiv. p. 464. 

80 Journal of Nervous and Mental Diseases, xxvii., 1900, p. 286. 



LEUKOSES ANT) PSYCHOSES 669 

concentric rings, processions of blue commas surrounded by green spots, 
while objects and persons were often surrounded by colored halos. I 
have recorded 91 a case of hysterical amblyopia associated with toxic 
amblyopia in which a marked symptom was xanthopsia. Ordinary non- 
chromatic visual hallucinations are met with in hysterics. Binet and 
others have shown that tactile sensations may be transformed in visual 
sensations in hysterical subjects. 

4. Organic Changes. — An essential condition of hysterical ambly- 
opia and amaurosis is the absence of organic lesions in the fundus of 
the eyes. Hyperesthesia of the retina may be present; enlargement of 
the retinal veins and serous exudate into the retina have been recorded 
hy Landolt. Leber and Nuel have described hyperemia of the retina and 
papilla, and a gray discoloration of the nerve-head which may eventuate 
in atrophy. But, as Nuel himself remarks, in such cases some other 
.agency should be suspected ; the hysteria may well have been associated 
with multiple sclerosis or tabes dorsaiis. The hysterical irido-choroiditis 
described by Simi is open to doubt. Hysterical amblyopia may simulate 
sympathetic disease, as has been well shown by Parinaud and Kalt. 92 
The latter author observed a young woman whose left eye had been 
destroyed by irido-choroiditis. There was depreciation of vision of the 
right eye, restricted fields for blue, and red and green achromatopsia. 
All of these symptoms disappeared when the left eye was enucleated. 
Hapid opacity of the crystalline lens after an hysterical attack has been 
Teported by Masleunikow. 93 He attributes the condition to nutritional 
disturbances caused by repeated cramp of the ocular muscles. An hys- 
terical vasomotor affection of the conjunctiva has been described by 
Eromaget 94 in the form of intermittent congestion of the bulbar con- 
junctiva without inflammatory phenomena. Hysterical oedema of the 
lids is probably always the result of some inflammatory condition of 
the conjunctiva or ciliary margin, and not an essential symptom of the 
disease. Some hysterics purposely bring irritating substances in contact 
with their eyes to cause congestion and excite sympathy, and may even, 
as did one of my patients, deliberately scratch their corneas with pins. 
Hysterical girls sometimes pull out their eyelashes, and to the result of 
this performance GifTord has given the name " hysterical alopecia of the 
eyelids." 

gi Ophthalmic Record, viii. 1899, p. 18. 
92 Annates d'Oculistique, vii. 1892, p. 37. 
93 Zeitschr. f. Augenheilk., v. 1901, p. 477. 
* 4 Recueil d'Ophtal., 3 Series, 24, 1902, p. 248. 






670 THE EYE AND NERVOUS SYSTEM 

CONCERNING THE ETIOLOGICAL FACTORS IN 
HYSTERICAL OCULAR STIGMATA. 

In general terms the psychoneurosis to which the name " hysteria" 
usually is applied is responsible for the development of the ocular signs 
which have been described, no matter which of the causes of the great 
neurosis may have been originally operative. As next to heredity 
trauma takes position as a cause of hysteria, it is not unnatural that 
many eye-symptoms of this disease form part of the symptomatology of 
traumatic hysteria. It is not germane to the subject to discuss the dis- 
tinction, if it exists, between hysteria and traumatic hysteria, to some 
phases of which, as is well known, under the name traumatic neurosis^ 
Oppenheim and Thomsen have given a special position in the nosological 
index, except to say that the ocular anomalies of both are analogous, if 
not identical, and in this respect, at least, if not in all others, the two 
affections are also identical. In the eyes the manifestations of hysteria 
which have been described as hysterotraumatic are often pronounced, — 
more so, frequently, than those which are associated with hysteria of 
other origins, — but they are not pathognomonic. In other words, given 
certain ocular stigmata of hysteria, one could not expect them to reveal 
the original cause of the hysteria, — that is to say, whether it depended 
upon heredity, trauma, disease, defective mentality, toxaemia, etc., — 
although they might vary in their severity and endurance. The eyes 
and, in general terms, the optical apparatus contain the necessary 
structures through which the well-known signs of hysteria — disturb- 
ances of sensation, spasms and contractures — may become evident in a 
manner similar to their development in other organs and regions of the 
body. 

To the disturbances of the functions of the eyes we are often able 
to apply tests more delicate than those which pertain to the investigation 
of other organs, and therefore should be able more accurately to inter- 
pret the symptoms. The admirable investigations of Landolt, Gale- 
zowski, Parinaud, Gilles de la Tourette, Pansier, Borel, Wilbrand, 
Schmidt-Rimpler, and Schwarz are of especial value in the thorough- 
ness with which their studies have been pursued from these and all 
other stand-points. 

Unfortunately, however, in many of the detailed journal reports of 
hysterical ocular phenomena the lack of ophthalmological training is 
only too evident, and especially regrettable is the absence of proper 
investigation of the refraction of the eyes. Thus, Dejerine points out 
that in many cases of so-called unilateral hysterical amblyopia it is 



LEUKOSES AND PSYCHOSES 671 

quite possible that the one eye may have been emmetropic, or approxi- 
mating that condition, while the other may have lost visual acuity 
owing to refractive anomaly which has wrongly been attributed to hys- 
teria. Pansier and Lapersonne insist on the difficulty of distinguishing 
between hysterical spastic convergent strabismus and convergent stra- 
bismus associated with hyperopia in an hysterical subject. The effect of 
hyperopic refraction on the anomalies of accommodation so commonly 
observed in hysterics must be evident, and Parinaud has not failed to 
call attention to it in his beautiful studies of this subject, and to the 
modifications which must necessarily be produced by myopia and pres- 
byopia. What the relations of the various phases of exophoria and 
esophoria must be to the hysterical anomalies of convergence is only too 
plain to the student of ocular muscle imbalance. In a word, there is 
constant danger of attributing to hysteria an anomaly of ocular func- 
tion, when really this is a condition belonging to the eyes of an hys- 
terical subject. More than this, — and it has been strangely neglected in 
the investigation of hysterical phenomena, — refractive error, especially 
astigmatism, muscular imbalance, in short, " eye-strain," are responsi- 
ble for many cases of hysteria which manifest themselves by well- 
known stigmata, not only in the eyes but in other regions and organs of 
the body, or by the phenomena pertaining to seizures. It may perhaps 
be doubted whether refractive error and heterophoria produce hys- 
terical manifestation in individuals of sound constitution and normal 
resisting power, but they certainly can call them into existence in 
those of neuropathic tendency. Given a normal subject with moderate 
hyperopic astigmatism and esophoria (convergence-excess), and the eye 
troubles will interpret themselves by headache, relieved by their cor- 
rection, or perhaps resisted without serious detriment for long periods 
without correction. In a neuropathic subject with the same defects 
their interpretation is likely to be spasm of accommodation, anaesthesia 
of the retina, contracture of convergence, restriction of the visual field, 
and areas of anaesthesia often relieved by their correction, but in the 
absence of it their influence is unresisted, and the long-enduring phe- 
nomena of what may be called a general hysteria result. 
For the treatment, see page 74. 

OCULAR MANIFESTATIONS OF NEURASTHENIA 
AND TRAUMATIC NEUROSES. 

Although the disturbance of the visual sensation to which neuras- 
thenic patients and those who are the subjects of traumatic neuroses are 
liable has been much investigated, it cannot be stated, as it was of 



672 THE EYE AND NEKVOUS SYSTEM 

hysteria, that such ocular signs as have been described constitute a dis 1 
tinct symptomatology. For convenience, the following subdivisions of 
the subject may be recorded : 

(1) Defective and painful visual sensation, or neurasthenic — more 
properly nervous — asthenopia. 

(2) Anomalies of the iris movements, or neurasthenic pupil phe- 
nomena. 

(3) Anomalies of the eyelid and eye-muscle movements, or so- 
called neurasthenic insufficiencies of the ocular muscles. 

(4) Anomalies of the visual field. 

(5) Previously unclassified neurasthenic ocular phenomena. 
Neurasthenic or Nervous Asthenopia. — Certain systematic writers, 

— for example, Wilbrand and Saenger, 95 — have made a distinction be- 
tween nervous asthenopia, or the insufficiency of visual power seen in ner- 
vous persons, as it occurs in school children and during the years of early 
development and as it appears in adults. School children, particularly 
from the ninth to the fifteenth year, often are brought for examination 
because they complain of a flickering before the eyes, — letters and 
figures disappear as they look at them and the print runs together, and 
reading creates lachrymation and severe brow and eye pain. When the 
child is asked to read he gradually approaches his face to the book, 
moves his head from side to side instead of following the lines with his 
eyes, and tries to bring to his aid stronger and stronger light. In 
association with the difficulty of vision are contraction of the eyebrows, 
half-closing of the eyelids, and often twitching of the face and muscles 
of the shoulder, or, in other words, the symptoms of the so-called habit 
spasm. This gradual approaching of the book towards the face, so that 
the patient is able to obtain larger retinal images, is more or less char- 
acteristic of this condition in childhood and is not seen in the adult 
cases. It depends upon a cramp of accommodation, and when the 
cramp subsides and there is tiring of the accommodative muscle there is 
corresponding blurring of the print and running together of the lines. 
If the visual power is tested in the ordinary manner with test-types, it 
will usually be found to possess a depreciation greater than would be 
accounted for by any error of refraction, and will often be promptly 
raised to normal if the letters are viewed through tinted glasses. In 
other cases glasses fail to improve the visual acuity. 

Associated with this asthenopia and diminution of visual acuity 



Ueber Sehstorungen bei functionellen Nervenleiden, Leipsic, 1892, p. 58. 



LEUKOSES AND PSYCHOSES 673 

concentric contraction of the visual field may be noted, in the manner 
which will be described in a subsequent paragraph. 

It is noteworthy that in children these ocular phenomena are marked 
to the exclusion of other neurasthenic phenomena, while in adults the 
reverse may be the case. The subjective symptoms in adults are similar 
to those just detailed. They have been described by Wilbrand, Standish, 
and others somewhat as follows : Headache, particularly throbbing in 
the brow and temples, occipital distress, pain in the back of the neck and 
spine, vertigo, muscse volitantes, defective accommodation, intolerance of 
light, and improvement in vision in the dusk and through tinted glasses. 
In addition to this, any attempt at concentrated vision is followed by a 
rapid disappearance from view of the object which is to be fixed. There 
are diminution of central vision, sudden attacks of obscuration of vision, 
processions of scotomas, visual hallucinations, lack of fixation of the 
optical memory images, persistent and confusing after-images, colored 
vision, — for example, erythropsia, — and a sudden red color of the pages 
of a book while the letters seem to be green. Associated with these 
phenomena are the characteristic changes in the visual field presently 
to be described. 

Etiology. — While it is true that neurasthenic patients, both children 
and adults, may possess as part of their symptomatology the symptoms 
of defective and painful visual sensation which have been detailed, 
nervous asthenopia, as Schwarz 96 properly remarks, is justified as a 
descriptive term only when the chief symptoms of asthenopia — namely, 
hyperopia, astigmatism, and ocular muscle imbalance — have been ex- 
cluded. In other words, as is now T well known but as evidently was not 
well recognized by earlier writers, these patients have nervous asthe- 
nopia not because they are neurasthenics, but because they have various 
refractive errors, particularly astigmatism, and various types of mus- 
cular imbalance, which, indeed, are in many cases the causative factors 
of the neurasthenia itself, and all of the symptoms, those referable to the 
eye as well as to the general economy, are simply the manifestations of 
eye-strain occurring in individuals of neuropathic constitution. They 
appear in these bizarre manifestations, because of such constitution, 
while in another individual of sound health they might either be resisted 
or find interpretation in a milder type of so-called asthenopia. 

Anomalies of the Iris Movements, or Neurasthenic Pupil Phe- 
nomena. — Unlike hysteria, there are no pupil phenomena which in any 



86 Die Bedeutung der Augenstorungen fur die Diagnose der Hirn- und Riicken- 
marks-Krankheiten, Berlin, 1898, p. 97. 
43 



674 THE EYE AND NERVOUS SYSTEM 

sense are characteristic of the neurasthenic state. It is true, as Beard 
pointed out, that the pupils are sometimes unequal, but not more 
frequently, as Schmidt-Rimpler 97 maintains, than has been observed 
under physiological conditions. Wilbrand also declares that the pupil 
reactions are usually normal, and that the slight differences in the 
width of the pupil which are often seen in these individuals depends 
upon certain conditions of the eye itself, and not upon the general and 
physical state. The inequality of the pupil observed in neurasthenia, 
according to Dercum, is always transient in character. It is true that 
the pupils of neurasthenics are often wider than those of more stable 
physical condition, and they sometimes seem to manifest an unusually 
prompt response to light impulse. On the other hand, occasionally they 
also act less promptly to light. 

Hippus is said to be more common in neurasthenia in an exaggerated 
condition than in other functional nervous states, but this is doubted by 
many competent observers, and my own observations of many neuras- 
thenics would not lead me to believe that hippus should be regarded as a 
sign of neurasthenia, nor have I ever seen any pupil change which is 
significant of a pure neurasthenia. Should there be reflex immobility to 
light in a patient exhibiting the symptoms of neurasthenia, there would 
be good reason to suspect tabes or beginning paresis. 

Anomalies of the Eyelid and Eye-Muscle Movements, or So-called 
Neurasthenic Insufficiencies of the Ocular Muscles. — In neurasthenia, 
unlike hysteria, anomalies of the musculature of the bulbus and of the 
lids are for the most part wanting. Certainly the various types of con- 
tracture of the eye muscles are lacking, nor is blepharospasm, as it has 
been described in connection with hysteria, present. A frequent symp- 
tom in neurasthenia is an inability of the patient to close the eyes 
completely when standing with the legs together (Romberg's sign), 
associated with marked fibrillary twitchings of the orbicularis. This lid 
phenomenon has sometimes been called RosenhacWs 98 sign, which he 
and Banas have considered as a characteristic symptom of neurasthenia, 
but which Schmidt-Rimpler declines to regard as peculiar to this affec- 
tion, inasmuch as it is often seen in healthy individuals. According to 
some writers, — for example, Loewenfeld, — this symptom is also present 
when the legs are separated or when the patient is in the sitting position. 
It has been ascribed to weakness of the orbicularis and to spasm of the 



97 Die Erkrankungen des Auges im Zusammenhang mit anderen Krankheiten, 
Wien, 1898, p. 276. 

98 Centralblatt f. Nervenheilkunde, ix., No. 17. 






NEUROSES AND PSYCHOSES 675 

sympathetic. As Knies puts it, the voluntary motor innervation is 
enfeebled and the sympathetic reflexes are increased. 

According to Landolt," there is a form of insufficiency of con- 
vergence which accompanies the neuroses, and especially neurasthenia, to 
which he has applied the term neuropathic insufficiency of convergence, 
and which he describes as follows: The excursion of the eyes may be 
normal, the action of the abductors — that is, of divergence — also normal 
or even augmented, but adduction is deficient. In such cases there is a 
reduction at both ends of the range of convergence. ( See also article on 
hysteria, p. 44.) Landolt believes that this insufficiency of the innerva- 
tion of the adductors, or of convergence, is a phenomenon which should 
be regarded as analogous to the weakness of the orbicularis which has 
just been described as Rosenbach's symptom. 

A symptom often noted in neurasthenic as well as hysteric patients 
(see section on hysteria, p. 656) is the so-called morning ptosis of Gowers, 
the night ptosis of Weir Mitchell, and which has been regarded by 
some authorities as a form of flaccid ptosis, which is, as has previously 
been noted, a frequent sign in hysteria. Certain nervous individuals, 
particularly women, usually on awakening in the morning, or during 
the night, find it impossible to raise their eyelids without assisting the 
action of the levator with their fingers. There is, in other words, a 
sensation as if the lids were glued together, and associated with it there is 
often a dry catarrh of the conjunctiva or a hypergemic condition of this 
membrane. I have studied a number of cases of night ptosis of this 
variety, and all of its subjects were nervous persons in the ordinary 
sense of the term and not hysterics. Usually they have been women past 
middle life, who have often been seriously alarmed, thinking that they 
had suddenly become the subjects of a paralysis. In most instances 
proper general regimen, with correction of existing refractive errors, 
has relieved the difficulty. Nystagmus has been described. According 
to P. C. Knapp, strabismus and nystagmus may be present in certain 
traumatic neuroses, but they are really traumatic scleroses. 

Anomalies of the Visual Field. — Of greater interest than the other 
ocular symptoms associated with neurasthenia are those changes in the 
field of vision which interpret the ready exhaustion to which fhe visual 
apparatus of the subjects of this condition are liable. They have been 



93 The Refraction and Accommodation of the Eye and their anomalies. Phila- 
delphia, 1886, p. 504. A System of Diseases of the Eye, edited by Norris and 
Oliver, vol. iv. p. 136. 



676 THE EYE AND KERVOUS SYSTEM 

elaborately and especially studied by Forster, 100 Wilbrand and Saen- 
ger, 101 Wilhelm Koenig, 102 Peters, 103 Frank-Hochwart and Topo- 
lanski, 104 Schmidt-Rimpler, 105 von Reuss, 106 H. Arnheim 107 and L. 
WolfTberg. 108 To the writings of these authors I am much indebted 
in the preparation of the following pages. 

In general terms these alterations in the visual field may be de- 
scribed as fatigue constrictions or contractions, and, according to Wil- 
brand and Saenger, the contraction may extend to the point of dis- 
appearance of the visual field, or, more usually, to a certain point of 
concentric narrowing. Eurthermore, the fatigue phenomena may appear 
in all of the four halves of the visual field or only in certain ones. 
According to the same authors, the signs of fatigue may manifest them- 
selves when the extent of the visual field and central visual acuity is at 
first normal, or when the extent- of the visual field is at first normal, but 
visual acuity is diminished, or, finally, when the extent of the visual 
field is concentrically contracted from the beginning and central visual 
acuity is diminished. 

Von Reuss insists that as neurasthenia is characterized by an easy 
exhaustion of the visual apparatus, this gives rise to visual fields of a 
fleeting and changing nature. The diminution of the size of the field 
appears, according to him, only during the process of examination, or, if 
it is present from the beginning, it is due to exhaustion of the eye by a 
previous examination. Systemic writers have described certain types 
of visual field fatigue as follows : . 

I. Concentric Contraction. — There may be a general concentric con- 
traction of the visual field similar to that seen in hysteria, but differing 
from it, according to von Reuss, because in neurasthenia it is constantly 



100 Bericht fiber die zehnte Versammlung der Ophthalmologischen Gesellschaft, 
Heidelberg, 1877, p. 162. 

101 Ueber Sehstorungen bei functionellen Nervenleiden, Leipzig, 1892, and Wil- 
brand, Die Erholungsausdehmmg des Gesichtsfeldes unter normalen und patholo- 
gischen Bedingungen, Wiesbaden, 1896. 

102 Ueber Gesichtsfeld-Ermiidung und deren Beziehung zur concentrischen Ge- 
sichtsfeldeinschrankung bei Erkrankungen des Centralnervensystems, Leipzig, 1893. 
(This work contains a full bibliography.) 

103 Deutsche Zeitschrift f. Nervenheilk., Bd. v., 1894, p. 302. 

104 Deutschmann's Beitrage zur Augenheilk., ii., 1895, p. 46. 

105 Die Erkrankungen des Auges im Zusammenhang mit anderen Krankheiten, 
Wien, 1898, p. 266. (This work contains an extensive bibliography.) 

106 Das Gesichtsfeld bei functionellen Nervenleiden, Leipzig and Wien, 1902. 

107 Fortschritte der Medicin, vol. xx., 1903, p. 44. (This paper contains a critical 
review of the subject with an analysis of the important papers.) 

108 Archives of Ophthalmology, xxxiii., 1904, p. 597. 



LEUKOSES AXD PSYCHOSES 



677 



changing during examination, while in hysteria it does not alter its size 
until recovery ensues. Moreover, there is no change in the sequence of 
the color circles. 

Temporary uniform concentric contraction of the visual field, so that 
it resembles a circle, or closely approaches in appearance a reduced 
visual field, frequently follows an hysterical, hystero-epileptic, or epi- 
leptic attack, and hence is not peculiar to neurasthenia. 

Permanent uniform concentric contraction of the visual field is not 
a neurasthenic phenomenon, unless those examples which Wilbrand has 
described in certain types of traumatic neuroses be excepted. 

II. Forster's Shifting or Displacement Type of the Visual Field. —Peri- 
metric examination of certain nervous patients will often yield a visual 
field of very irregular type, due to a more or less unsymmetrical contrac- 
tion of the field from meridian to meridian brought about by fatigue. 
This is the important symptom of that condition called by Yon Graefe 




Fig. 12. — Forster's shifting or displacement type of the visual field. (After Wilbrand. ) 

" anaesthesia of the retina," but now generally known by the name of 
" nervous asthenopia," proposed by Wilbrand in place of the more re- 
stricted term " neurasthenic asthenopia," and the general visual symp- 
toms of which have already been described. (See Page 672.) 

Forster 109 was the first to examine the fatigue phenomena of the 
visual field under these circumstances with particular care. His method 
is as follows: The test-object is carried on the perimeter from the 



Loc. cit. 



678 THE EYE AND NEKVOUS SYSTEM 

periphery of the temporal side through the fixation point to the nasal 
side along all the meridians, and its points of entrance and exit noted on 
the chart. It will be found that the visual field obtained has a greater 
extent in the temporal half, — that is, the side where the test-object first 
entered the field, — than on the nasal side, because when the latter was 
tested the patient was already fatigued. If after an interval of rest the 
central test is made by examining the same meridians in the same order, 
but now starting from the periphery of the nasal side, and the points of 
entrance and exit of the test-object noted on the chart, it will be found 
that the visual field is greater in extent on the nasal half because it is the 
less fatigued half of the field. In other words, two visual fields are 
obtained, the outlines of which appear to have shifted or been displaced 
in their relation to each other. For this character of visual field 0. 
Koenig suggested the term " shifting or displacement type," while 
Schiele described the procedure by the name " diametrical method." 110 

Schiele (quoted by Wilbrand) devised a method of investigating the 
field of vision to which the name radial centripetal method has been 
given. His investigations were made with a Scherk's perimeter. The 
meridians were systematically fatigued, only in one-half of the visual 
field, from the periphery as far as the fixation point, and he found in 
certain cases that an equally large fatigue defect was found on the 
same meridians of the homonymous halves of the visual field of the 
other eye, which had been covered during the previous examination, as 
on the half of the eye which had been examined first. To his explana- 
tion of this phenomenon we shall return later. 

III. Wilbrand's Exhaustion Type of the Visual Field. — Because For- 
ster's method is time-consuming and very fatiguing to the patient, Wil- 
brand materially simplified it, confining his examination to the hori- 
zontal meridian. Von Eeuss, however, declines to consider Wilbrand's 
method as a simplification of the plan pursued by Forster and prefers to 
give it a special classification, suggesting the title " Wilbrand's exhaus- 
tion type." According to Wilbrand, fatigue phenomena, whenever pres- 
ent, occur on all of the meridians of the corresponding half of the visual 
field. Hence, in order to determine the defatigability of the nervous 
system quickly, he proceeds as follows : He uses the horizontal meridian 
as being the meridian of the visual field of greatest extent. Beginning 
at the temporal edge of the perimeter, he carries the white test-object 
along the horizontal meridian with as steady a movement as possible in 
the direction of the arrow (Fig. 13) toward the nasal half, and has an 

110 Consult Wilbrand and Koenig, loe. cit. 



NEUROSES AKD PSYCHOSES 



679 



assistant mark on the diagram with a O the point at which the white 
test-object enters the field and with a I the point where it disappears. 
Having reached the point I on the nasal half, the direction of the test- 
object is at once reversed and it is carried with the same uniform 
velocity along the horizontal meridian toward the temporal half. The 
point on the temporal half of the horizontal meridian where the test- 
object again disappears after its second fatigue excursion is recorded on 
the diagram by the figure 2. The object is then at once brought back 
again in the opposite direction and the point at which it disappears on 
the nasal half indicated by the figure 3, and so on. 

This method of examination takes little time, and according to Wil- 
brand, enables the examiner to recognize whether concentric constriction 
of the visual field was already present at the beginning of the examina- 




Fig. 13.— Wilbrand's exhaustion type of the visual field. (After Wilbrand.) 



tion, and whether there was with it a slight degree of def atigability ; on 
which half of the visual field this defatigability is chiefly elicited, and 
whether it can be extended as far as the fixation point or ceases at a 
definite parallel on the perimeter. 

To these advantages (quoted from Wilbrand) W. Koenig adds 
another, namely, that the method affords an accurate means of measuring 
the patient's attentiveness, or, in other words, his ability to give ser- 
viceable answers when examined with the perimeter. 

The portion of the visual field which resists fatigue has been called 
by Wilbrand "minimal visual field." Analogously W. Koenig desig- 
nates the visual field as it was in the beffinnino; of the " maximal visual 



680 



THE EYE AND NEKVOUS SYSTEM 



field. " According to Wilbrand, whenever fatigue is present, it appears 
on all the meridians of the same half of the field, and the fatigue of the 
visual field is the same for colors as for white. W. Koenig, however, 
thinks that while the visual field becomes fatigued for white, it does 
not lose its reactions to colors at the same time. Eor example, if a visual 
field has been fatigued down to the minimal visual field by means of a 
white test-object, if a colored test-object is brought into the field from 
the temporal side without any interval of rest, the point of entrance of 
the said colored test-object approximately coincides with the point where 
it is first seen after a considerable period of rest. Sometimes, according 
to Koenig, only a slight difference can be demonstrated, and if no inter- 




Fig. 14.— Oscillating visual field. (After Wilbrand ) 

val of rest is allowed the limits obtained are narrower. The same 
behavior to white and the remaining colors is observed in the visual field 
if a colored test-object has been used in producing the fatigue. 

IV. The Oscillating Visual Field and Fatigue Scotomas. — According to 
Wilbrand, in contrast with types of constant progressive fatigue, cases 
are occasionally observed in which, to quote his own words, the fatigue 
pursues, so to speak, a ryhthmical course, the test-object vanishing from 
time to time along the meridians, to emerge again after a short interval 
and again disappear and emerge, and so on. For this condition 
Wilbrand suggests the term oscillating visual field. Tie believes that 
the condition represents an alternating flaring up and extinguishing of 
the optic sensory tracts. The same oscillation obtains when a colored 
object is used. Such visual fields, which are not common, have been 
observed by him and others, particularly Koenig, in various nervous 



LEUKOSES AND PSYCHOSES 



G81 



conditions, — that is, not only in post-epileptic states, but in neuras- 
thenic patients. 

These authors and others have also described temporary central 
scotomas representing fatigue phenomena of the macular portion of 
the visual field, especially frequent in young persons of neurasthenic 
tendency. The scotoma may be confined to the fixation point or extend 
beyond it. It is chiefly manifest to small colored objects, less commonly 
when the test-object is white. It will be remembered that in the section 
on hysteria similar phenomena were mentioned, and Von Reuss believes 
these types of oscillating visual field and scotomas are really hysterical 
manifestations. 

V. Fatigue Spiral Field (Exhaustion Spiral). — In ordinary perimetry, 
the eye being normal, if the points on each meridian at which the test- 
object is first seen are united with a line, the well-known shape of the 
visual field appears. If this examination is repeated, an exactly similar 
map will be produced, or one which differs only slightly from the origi- 




Fig. 15.— Fatigue spiral field, (von Reuss.) 

nal. If, on the other hand, according to von Reuss, 111 fatigue of the vis- 
ual field develops, then, the first field having already been taken, the 
point at which the test-object in the second trial appears in the visual 
field on the horizontal meridian, for example, lies nearer to the centre, 
and this is also the case in all meridians. When the examiner begins for 
the third time, the point of appearance of the test-object lies still nearer 
to the point of fixation, and so forth, until further contraction can no 
longer result and the later points of appearance of the test-objects coin- 
cide with the earlier ones. If all of these points are connected, a spiral 



Loc. cit., p. 64. 



682 THE EYE AND NERVOUS SYSTEM 

line is formed, which ultimately terminates with a closed line. To this 
form of the visual field von Eeuss has given the name " fatigue spiral." 
It goes without saying that the visual field does not possess the form of a 
spiral, and that the reduction in size does not take place in such a form. 
The narrowing takes place because the boundaries are contracted con- 
centrically and the points at which the test-object is first noticed are 
only the stages at which the boundary lines cut the individual meridians, 
at the moment when the test-object appears. These, connected by lines, 
take the form of a spiral, a snail's house, or a watch-spring. Similar 
fields, according to this author, have been noticed by Straub and Fuchs. 
He thinks the spirals are not more frequently observed because they are 
not properly searched for. 

VI. The Recuperative Extension Type of Visual Field. — The contracted 
visual field may enlarge after recovery from fatigue. Necessarily the 
rapidity of recuperation depends upon the previous degree of fatigue. 
Yon Reuss thinks that such enlargement is particularly easy to observe 
in connection with the spiral fields. Such enlargements, for example, 
have been noted during psychic disturbance and as the result of will 
power, and thus explain the negative spirals of Fuchs, who demon- 
strated graphically the successive dilatation of the visual field under 
these circumstances. Recovery of the fatigue of the visual field may be 
produced by long-continued rest, especially if the subject remains in a 
dark room and all irritations of the optic nerve are removed. As is 
well known, Wilbrand has studied this matter particularly and examined 
the visual field in the dark, using illuminated colors. As von Reuss 
points out, interesting as are his results, the examinations are difficult 
and often yield contradictory results. 

VII. The Mixed Forms of Visual Field. — Reference has already been 
made to these in the section on hysteria. Von Reuss contends that all 
constant visual field contractions are of hysterical nature, while every- 
thing which is of variable nature in the visual field belongs to neuras- 
thenia. Contracted visual fields, he believes, which become still smaller 
in the course of examination, belong to the mixed forms. This mixture 
of hysteria and neurasthenia is not uncommon, especially in trau- 
matic neurosis. According to him, the visual field under these circum- 
stances gives evidence of* the mixture of the two nervous conditions, the 
constant concentric limitation indicating hysteria and the exhaustion 
spiral neurasthenia. Naturally, all precautions must be taken to elimi- 
nate organic disease of the eye. 

Pathogenesis of the Fatigue Field.— The exact nature of the 
fatigue which brings about the various forms of visual field which have 
thus far been described is still the subject of much discussion. The 



NEUROSES AND PSYCHOSES C83 

whole matter has recently been critically reviewed in an admirable 
manner by H. Arnheim, 112 and from his paper the following interesting 
views are quoted : According to Wilbrand, concentric and fatigue con- 
traction of the visual field are interpretations of the same affection, 
— namely, insufficiency of the retina, — the entire symptom-complex of 
nervous asthenopia, according to him, in so far as it relates to alterations 
and insensitiveness to light, is localized in the external layer of the 
retina, where it is dependent upon changed conditions of metabolism. 
Through the mediation of the optic tracts, however, probably centrifugal, 
these metabolic processes may come under the influence of remote por- 
tions of the brain. 

Peters regards the narrowing of fatigue as representing a disturb- 
ance of innervation which transmits stimuli from every point of the 
retina to the two optic tracts. Any disturbances in the nervous paths 
which subserve this function produce displacement types of the visual 
field, which accordingly represent a relative insufficiency of the optic 
nerve. 

Placzek 113 contends that the displacement types of the visual field 
depend upon a diminution and blunting of the centre of consciousness — 
that is, a diminution in the respective power of the centre. 

According to Simon, 114 the phenomena of fatigue should be referred 
to a physical basis. The forms in which fatigue ceases after the first 
examination are ascribed to a diminished power of psychical perception 
as compared with sensory irritability, and those which increase as the 
examination proceeds are supposed to depend upon a general fatigue of 
the psychic sphere. In a word, Simon believes that these phenomena 
are due to an exhaustion of the psyche. This theory von Reuss also 
adopts as being more comprehensive than the others. 

Finally, we come to the views of those who do not regard the con- 
traction of the visual field depending upon fatigue as a pathological 
process, but as an effect of a disturbance of concentration. In other 
words, such conditions can be brought about by inattention and lack of 
concentration, and inattention, according to Schmidt-Rimpler, best ex- 
plains the phenomena which have thus far been described. 

Wolfiberg 115 points out that in practice usually the suspicion of 
traumatic neurosis or allied condition is entertained before the visual 
field examination is begun, and hence the various types of fatigue field 

112 Fortschritte der Medicin, vol. xxi., 1903, pp. 44-54. 

113 Berlin, klin. Wochenschr., 1892. 

114 Arch. f. Opth., xl., Bd. 4, 1894. 

115 Archives of Ophthalmology, vol. xxxiii. 1904, p. 001. 



684 THE EYE AND NERVOUS SYSTEM 

are searched for without examination of the field in the usual manner. 
But he does not believe with Schmidt-Rimpler that contraction of the 
field in neurasthenic patients by fatiguing examinations is wholly due to 
relaxed attention. He thinks, moreover, that von Reuss has given ex- 
perimental proof that the fatigue of the neurasthenic is chiefly a psy- 
chical fatigue. As the fatigue is psychical, he maintains it is also mani- 
fest in direct vision, and he regards slight fatigue of the macular region 
as a symptom of traumatic neurosis not less important than that of 
peripheric vision, and that each is connected with the other. 

Schiele's theory (his results in visual field examinations under these 
circumstances having already been referred to) that each individual 
portion of one retina is in relation with the homonymous portion of the 
other, and that fatigue of the retina must be regarded as fatigue of the 
cortex of the occipital lobe, has been practically rejected, although at 
one time Wilbrand thought that it was worthy of attention. It has 
been disputed largely because Groenouw and other observers have been 
unable to reach the same results which he did in visual field examina- 
tions, — that is, they could not confirm his contention that only homony- 
mous parts of the two visual fields show contraction, because they found 
always when one sector of the visual field was fatigued there were 
changes in the outer limits of the remaining portions of the visual 
field of the same eye. 

Concerning the Diagnostic Value of Fatigue Fields. — As Wilbrand 
has already, pointed out, when he adopted the term " nervous asthe- 
nopia" in place of " neurasthenic asthenopia," fatigue fields may 
accompany any form of nervous disease. They are, therefore, not 
peculiar to or diagnostic of neurasthenia. Moreover, they are not con- 
fined, as Schmidt-Rimpler has said, and as the investigations of Peters 
and Voges show, to nervous individuals, but may be found in healthy 
persons. As we know, and as Arnheim points out, contractions of the 
visual field may be found in diseases without definite anatomical foun- 
dation, — for example, in epilepsy, trifacial neuralgia, Basedow's disease, 
heart disease, chlorosis, alcoholism, etc. Finally, it must not be for- 
gotten that the contracted visual field may be the result of malingering. 
On this point something more will be said in a future paragraph. 

All things being taken into consideration, however, and not denying 
the foregoing facts, it would seem proper to agree with Arnheim that 
the value in diagnosis of hysteria and neurasthenia of contracted visual 
fields is undoubted, although in many complex cases it is frequently 
difficult to decide what the exact cause of the narrowing may be. 
Whether one can make such a sharp differentiation as von Reuss does,. 



NEUROSES AND PSYCHOSES 685 

that fields which do not change during examination and do not mate- 
rially differ under control, indicate hysteria, while fields which at first 
are normal but change into the various fatigue types during examination 
indicate neurasthenia, and that an initial diminution of the field which 
is the consequence of exhaustion but which goes on increasing during 
examination indicates hystero-neurasthenia, I am not prepared to say, 
but of the definite value of these examinations in such cases and their 
importance as indicators of a search for other symptoms of the condition 
there can be no question. 

Wolff berg 116 considers the field of examination important. Accord- 
ing to him, fatigue in traumatic neurosis is nothing other than dimin- 
ished neuroptic excitability, and the diagnosis secured by finding various 
exhaustion types of the field can also be reached by determining con- 
traction of the color fields. Furthermore, even more important than 
the condition of the general field is that every part of the field, even 
that corresponding to the fovea, is similarly affected by fatigue. Reduc- 
tion of the excitability of the fovea can be ascertained by making tests of 
color perception in the distance. He concludes that a field taken in 
ordinary daylight, which in its limits for white and colors corresponds to 
those obtained with a normal eye in diminished illumination, is typical 
of diminished excitability of the neuroptic apparatus and of abnormal 
fatigue and hence of traumatic neurosis. 

It must always be remembered that neurasthenics often exhibit no 
change in their visual fields, and the same is true of hysterics. Indeed, 
some authors, — for example, Berger and Topolanski and Hochwart, — 
almost never found any changes in the visual field which they could con- 
sider as part of the neurasthenic process. 

The tendency at the present time, as Schmidt-Rimpler says, is to 
speak of traumatic hysteria, traumatic neurasthenia, and traumatic 
hypochondriasis, instead of using the general term, traumatic neurosis. 
Hence, no attempt has been made to separate the visual phenomena 
which pertain to pure neurasthenia from those which belong to the 
traumatic variety of the affection, because, as Oppenheim says, the phe- 
nomena of accident neuroses, in so far as the visual field examinations 
are concerned, and in general terms, the visual symptoms, are, to a great 
extent, identical with those of neurasthenia, hysteria, and hypochon- 
driasis. Just as the hysterical visual stigmata are often more pro- 
nounced when traumatism has been the etiological factor, so also the 
ocular signs of fatigue, whether they interpret themselves by the general 

116 Loc. cit., p. 206. 



686 THE EYE AKD NEKVOUS SYSTEM 

symptoms of nervous asthenopia, or by the symptoms which are par- 
ticularly elicited by mapping the visual field, are often more decided. 

Concerning the differential diagnosis from the ocular stand-point of 
hysteria and neurasthenia enough has been said in the preceding pages 
and need not again be summarized in the present paragraph, except 
to reiterate that hysteria presents certain definite ocular stigmata, while 
those which arise in the course of neurasthenia cannot be said to be 
peculiar to it, but represent forms of visual fatigue, which may be 
produced by a variety of nervous affections, although when taken into 
consideration with other signs, they have their definite place in the 
symptom-complex of this affection. 

As John K. Mitchell and myself 117 have stated, in the difficult dis- 
tinction between certain types of neurasthenic and hysteric patients, the 
presence of the disturbance in the color-sense is of some diagnostic im- 
port. It is less apt to be present in the former than in the latter, and yet, 
as we have seen, its absence is of little meaning. It may not be found in 
many typical cases of hysteria and seems to be present in others which 
are difficult to eliminate from the neurasthenic category. Yon Erankl- 
Hochwart and Topolanski, who, as before pointed out, believe that 
neurasthenic patients rarely exhibit concentric contraction of the field,, 
and that their light-sense and even visual acuity are usually normal, 
have noticed a certain tendency of lowering of color perception, particu- 
larly in connection with blue. If this should be substantiated, it might 
be considered as of some diagnostic importance. Wolffberg's view with 
reference to colored central color perception has been referred to (pages 
683 and 685). 

Previously Unclassified Neurasthenic Ocular Phenomena. — Cer- 
tain systemic writers have described a neurasthenic conjunctivitis, 
which, however, more properly should be regarded as a hyperemia of the 
conjunctiva in persons of neurasthenic condition, and which, owing to 
their nervous instability, even though it be moderate in degree, presents 
to them a reality far in excess of its real importance. It is probable that 
this may account for the well-known neurasthenic symptom of foreign 
body sensation in the eye, sometimes associated with drooping of the lid, 
to which reference has already been made, a condition which exactly 
simulates eye-strain and which often is totally unrelieved by the adjust- 
ment of suitable glasses, but disappears when with the wearing of these 
the proper physical and mental hygienic measures are adopted. The 
same is true of the pricking and burning sensation so frequently com- 
plained of by these patients and located along the margins of the lid. 

117 Journal of Nervous and Mental Diseases, January, 1894. 



NEUROSES AXD PSYCHOSES 687 

Ophthalmoscopic phenomena are wanting, or if they are present in 
the form of various hypersemic conditions of the eye-ground and con- 
gestions of the margins of the optic disc, indicate the signs produced by 
ametropia. 

A rare condition is temporary exophthalmos, particularly when the 
patient stoops over, which has been described by Oppenheim. 

TREATMENT OF THE OCULAR DISORDERS OF 
HYSTERIA AND NEURASTHENIA. 

Naturally, treatment from the general stand-point is indicated, 
and as this belongs to the domain of the neurologist and general 
physician, it need not be discussed here, but the eye-treatment of itself 
is of the greatest importance. First and foremost, there should be the 
most thorough investigation of the refraction of the eyes, and search 
made for the smallest degrees of astigmatism. Suitable glasses should 
be adjusted after the thorough employment of atropine or other suitable 
cycloplegic, and they should be worn constantly. Anomalies of the 
ocular muscles require similar careful consideration. Personally, I 
believe that insufficiency of convergence should be treated by suitable 
gymnastic exercises with prisms, and I do not believe, a few cases ex- 
cepted, that the constant wearing of prisms, laterally placed, is a wise 
procedure. Prisms, base in, associated with reading-glasses, may be 
required. On the other hand, the symptoms produced by the various 
types of hyperphoria sometimes yield rapidly if vertical prisms are 
combined with the correcting lenses, or if a like result is produced 
by proper decentration. This is not the place to discuss elaborately the 
propriety of surgical interference. I have often expressed my belief 
in regard to this matter. I hold that surgical interference is to be 
considered only when all other measures have been long and faithfully 
tried and have failed to give relief. While cases of muscular imbalance, 
best treated by operative interference, are encountered (aptly called by 
Risley, absolute insufficiencies, equivalent to the structural and inser- 
tional anomalies of Duane), in my opinion they represent a limited por- 
tion of the whole number. ^Moreover, as our knowledge of the etiology 
of the abnormalities of muscular balance increases, our methods of non- 
surgical treatment improve, and this number grows steadily smaller. 

The point, however, should be emphasized, that refractive errors 
and anomalies of muscular motility are frequently the cause of the entire 
hysteric and neurasthenic outbreak, and therefore should be among the 
first problems to be carefully investigated in the management o^[ these 
cases. This does not mean that ocular treatment should be utilized to 



688 THE EYE AND KEKVOUS SYSTEM 

the exclusion of general measures. To maintain this would be absurd, 
but it is equally absurd to attempt cures in many of these cases without 
consideration of the constantly associated disturbances of refraction, 
accommodation, and muscular balance. Moreover, it is a distinct mis- 
take to put these patients through long periods of so-called rest-cure, as 
William Thomson long ago pointed out, without the relief which neces- 
sarily must come from the restoration of normality to the eyes. I do 
not doubt that many a rest-cure would be materially shortened if this 
plan were adopted. Because a patient lies in bed or rests in his room, 
there is no reason why he should continue to submit to the plague of 
■eye-strain, which, while it may not be as great as it is in the presence 
•of sustained close eye-work, is constantly in evidence so long as ametropic 
veyes are utilized in the effort of sight. 

This has been fully recognized for years by many neurologists, and 
accounts for many brilliant results which have been obtained in the mod- 
ern treatment of functional nervous diseases. Unfortunately eye-strain 
lias been brought into etiological relation with a number of nervous 
diseases with which it has no -such concern by certain injudicious 
writings, and consequently some physicians have been led to believe 
that its influence has been exaggerated. This deduction is regrettable 
.and has induced them to neglect the potency of eye-strain in the pro- 
duction and fostering of many neurasthenic and hysteric phenomena. 

Because photophobia is a frequent symptom in functional nervous 
•diseases, tinted glasses have been recommended. They should be used 
with caution, although in some cases excellent results follow the adjust- 
ment of lenses which correct the refractive error, and which have been 
slightly tinted or manufactured of Arundel or lightly smoked glass. 
Division of the supraorbital nerve to relieve blepharospasm has been 
recommended (von Graefe, Panas) and practised. It would seem to be 
an unnecessary procedure and probably acts, as Parinaud says, by sug- 
gestion. Indeed, many of these patients readily accept suggestion, and 
may at times be relieved by this means, hence the success of Christian 
Science and certain " mind cures." For the same reason magnets have 
been employed and their use followed by the disappearance of the 
amblyopia and other signs of ocular hysteria. 

THE OCULAR MANIFESTATIONS OF HYPOCHONDRIASIS. 

There are no ocular symptoms characteristic of hypochondriasis. On 
the other hand, some perfectly trivial ocular complaint may be one 
upon which the morbid fancy of the patient centres; for example, a 






NEUROSES AND PSYCHOSES 689 

mild conjunctivitis or a recurring hyperemia of the conjunctiva may 
cause him to go from one consulting-room to the other, hoping for relief 
and believing in all manner of evil consequences. The same is true if he 
once becomes conscious of the shadows which the formed elements of 
the vitreous cast upon the retina and to which we give the name muscse 
volitantes. They are not caused by hypochondriasis nor are they a 
symptom in one sense, but they are a condition which may greatly 
aggravate the mental disturbance. Long-standing hypochondriasis may 
produce contraction of the visual field, and phenomena which have been 
described in general terms as fatigue fields, or exhaustion fields, just as 
they occur in neurasthenic patients. 

A matter of some importance is the effect of presbyopia on these 
patients. It seems to me that there is no doubt, and I find that this is 
the opinion of some accurate neurologists, that neglected presbyopia or a 
failure of accommodation, for which no adequate relief is sought, even 
before the presbyopic age, is frequently the cause of hypochondriasis 
and mild forms of melancholia. The patients cannot be made to 
understand that it is the physiological failure of accommodative power, 
and believe that some form of visual disturbance is arising which later 
will eventuate in blindness. The irritating inconvenience of oncoming 
and increasing presbyopia doubtless aggravate these mental conditions 
under many circumstances. That eye-strain in general may produce 
hypochondriasis is well known. 

OCULAR MANIFESTATIONS OF HYPNOTISM AND SLEEP. 

Pansier 118 has devoted a chapter to the ocular conditions during 
hypnosis and the action of suggestion on the visual function. 

During hypnotic sleep the eyelids are usually closed. They can, 
however, be found partly or sometimes completely open, and the eyes 
may be rotated upward and the pupils dilated and sluggish. 

Although some writers have stated that hypnotic hyperesthesia may 
be concerned with all the senses except the sense of vision, this is evi- 
dently not an accurate statement, and more than one author has observed 
increased visual power in the hypnotic state, even when the lids seem 
closed, because the patient looks through a scarcely perceptible slit in 
the palpebral fissure. Pansier maintains that there is a hypera?sthetic 
condition of the retina, which under these circumstances has the effect of 
rendering it more sensitive to the rays of light. The same author quotes 

118 Les Manifestations oculaires de l'Hysterie. Oeil Hysterique. Paris, 1S02. 
p. 31. 

44 



690 THE EYE AND NERVOUS SYSTEM 

Binet as having observed in a subject passing from the normal to the 
hypnotic state a decided augmentation of visual acuity. 

Koenigshof er x 19 states that in so far as accommodation is con- 
cerned for vision at a distance, there appears to be a relaxation of the 
ciliary muscle. In effect the hyperope seems to have accentuated 
his ametropia, the myope, on the contrary, to have diminished it. Now 
it is an interesting fact first noted by Heidenheim that spasm of accom- 
modation is one of the first signs of beginning hypnosis, and therefore 
distant vision would be diminished because of the fictitious myopia 
which is produced. Hence, should there be at a later stage of the hyp- 
nosis, relaxation as has just been stated, of the muscle of accommodation, 
the patient would suddenly find after his return to consciousness, par- 
ticularly if he was a hysteric, that the visual acuity was suddenly 
augmented. Pansier quotes Sciamina and Parisotti as having found 
in their observations a constant augmentation of refraction. 

Knies, on the authority of Tamborini and Sepilli, states that the 
pupil during the cataleptic state of hypnosis is dilated and doas not 
react to light, and quotes Fere as describing dilation of the pupil 
during cataleptic sleep if the individual is directed to look at a distant 
object, but contraction of it if he looks at a near object. This has been 
proved to be the case in a number of other experiments in hypnotism. 

According to Koenigshofer, pupillary reflexes are produced more 
slowly in the hypnotic than in the normal state. Berger has caused 
dilation of the pupil after strong sensory irritation in the most pro- 
nounced hypnotic sleep. 

A number of observers have studied the visual field during the 
various phases of hypnotism and have found a dilation of the visual 
field in the somnambulistic stage. Pansier records a number of in- 
stances of this character, — for example, a marked restriction of the 
visual field during waking, while during hypnosis it widened out 
almost to normal. The visual field for colors in one of his cases was 
also enlarged, but the colored circles (the patient was a hysteric) pre- 
served their characteristic place and inversion within the limits of the 
field. 

Moravcsik, 120 after pointing out that a contracted visual field may be 
much widened in hysterical patients by certain irritations, — for example, 
pouring ether on the hand, touching the tongue with salt, sounding a 
loud tuning-fork in the ear, — demonstrated in a hypnotic patient 



119 Klin. Monatsbl. f. Augenheilk., xxvi., 1888, p. 13. 

120 Neurolog. Centralbl., 1899, p. 230. 



NEUROSES AKD PSYCHOSES 691 

that the width of the field may vary according to suggestion. I have 
studied the visual field during hypnotism, and in one case found it 
markedly contracted. 

The ophthalmoscopic conditions during hypnotism have been investi- 
gated by several observers. In the beginning of hypnotism Forster 
found the fundus normal. I have had only slight opportunities of 
making observations under these conditions, but in Weir Mitchell's 
clinic in the Orthopaedic Hospital have not found any alterations of 
significance, although, as is well known, in the later stages of hypno- 
tism, as particularly has been demonstrated by the researches of Luys 
and Bacchi, the retinal veins may be much dilated, and this state of 
passive hyperasmia persisted during the stage of catalepsy. The same 
phenomena were observed in the phase of fascination. In the cataleptic 
stage the iris was immobile ; in the somnambulistic stage it reacted to 
light, and at this time the arteries and veins were increased in volume. 

In so far as the action of suggestion on the visual function is con- 
cerned, it may be stated that all the symptoms of hysteria may be pro- 
duced by suggestion, and in general terms it may be said that both 
binocular and monocular blindness has been produced by suggestion, as is 
especially evidenced by the investigations of Fontan. The dilatation of 
the visual fields which occurs under the same condition has already been 
referred to. Koenigshofer by his experiments confirmed the statement 
that hypnosis can produce amblyopia and amaurosis and all hallucina- 
tions of vision, but the opinion maintained by several authors that color- 
blindness can thus be produced is, according to him, an error. He 
maintains that it is not the impression produced by the color which is 
neutralized, but it is the designation of the color, — that is, the idea of 
red or green, for example, which is abolished. 

As already pointed out, the condition of the pupil and accommoda- 
tion varies according as the suggestion is made to the subject of viewing 
objects which are near or far away. This augmentation of accommo- 
dation has been proved by Pansier by keratoscopic examinations to occur 
during suggestion to the subject that he shall look at some object close at 
hand, while if the suggestion is made that the object is now removed 
to a long distance, there is relaxation of accommodation and a replace- 
ment of the visual axes from convergence to parallelism. 

The action of suggestion on the ocular muscles and the artificial 
reproduction of contractures of the muscles has been studied by Fontan 
and particularly by Borel, and in this manner ptosis, strabismus, both 
convergent and divergent, have been brought into existence. Divergent 
strabismus is much more difficult to produce than the convergent variety 



692 THE EYE AND NERVOUS SYSTEM 

of the affection, and it is said that even an immobilization of the eye in 
the condition of conjugate deviation has been created by similar methods. 

The condition of the eyes during ordinary sleep may now receive 
brief notice. Sleep begins with closure of the lids, or, in other words, 
with ptosis. This sleep-ptosis Mauthner regarded as representing a 
temporary nuclear paralysis of the levator. Inasmuch, however, as 
there is relaxation of the tonus of the sympathetic fibres which pass to 
the blood-vessels during sleep, Knies and other observers consider that 
the drooping of the lids is also an evidence of a paresis or, at least, an 
insufficient action of the sympathetic nerve. This is further confirmed 
by the fact that during sleep the pupils are contracted, while when the 
lids are simply closed during waking they are dilated. In other words, 
together the two symptoms of drooping of the lids and contraction of the 
pupils would produce the phenomenon, as Knies puts it, of sympathetic 
ptosis with myosis. 

The visual axes are divergent — that is to say, during sleep there is a 
relaxation of convergence-innervation — and the eyes, to use the language 
of Hansen-Grut, take up their anatomical position of rest. 

Ophthalmoscopic examination of sleeping patients would be ex- 
tremely difficult to make except under unusual conditions. So far as I 
am aware, nothing of interest has been developed in this line of examina- 
tion, although it is possible that there might be some change noted in the 
calibre of the retinal vessels. 

TESTS FOR DETECTING PRETENDED AMBLYOPIA 
(MALINGERING). 

In order to investigate the various amblyopias which occur in hys- 
teric patients it is necessary to apply the tests which are ordinarily used 
for detecting malingering. To a certain extent they have been referred 
to in the preceding pages. The subject has been especially investigated 
by Professor S. Baudry, 121 to whose writings those interested in the 
great variety of tests which have been devised and which often are 
extremely useful, especially in medico-legal cases and in work connected 
with the examination of recruits for the army and navy, should turn. 
Evidently for practical purposes it is sufficient to briefly describe a few 
that are in common practice. 

The information which may be gained by testing the pupils in their 
reactions is elsewhere described (see Chapter VI), and we are therefore 
concerned for the moment with simulated blindness of one eye, simu- 

121 Simulated Blindness, System of Diseases of the Eye, edited by Norris and 
Oliver, vol. iv. p. 861. 



• NEUROSES AND PSYCHOSES 693 

lated blindness of both eyes, and simulated contractions of the visual 
field. 

Simulated Blindness of One Eye — To detect this condition the fol- 
lowing methods may be used : 

(1) The Diplopia Test. — This is performed in the usual manner of 
the ordinary examinations of the external ocular muscles at 5 metres, 
and a 7 degree prism is placed before the admittedly sound eye. If 
now superimposed double images are acknowledged, there is binocular 
vision and the fraud is detected. The examiner may vary the test by 
placing the prism before the supposed blind eye, either base up or 
base down. 

(2) Harlan's Test. — This is an extremely useful and simple device 
and is performed as follows : Place in position an ordinary trial frame 
and put before the admittedly sound eye a high convex glass (-f- 16D) 
and before the eye which is claimed to be blind a plane glass or a weak 
concave spheric ( — .25D), which will not interfere with vision. If 
the proper letters placed at a distance of 5 metres are read, the act of 
reading must have been done by the eye which was claimed to be sight- 
less, inasmuch as vision at that distance with the other eye is excluded 
by the presence of the high convex lens. The test may be further 
elaborated by covering the pretended blind eye and requesting the 
patient to read the letters. If he is unable to do so the fraud is exposed. 

(3) Baudry's Test with Monocular Diplopia. — Inasmuch as Graefe's 
method of producing monocular succeeded by binocular diplopia by 
means of a prism is rather difficult of application, Baudry has invented 
an apparatus which consists of a prism supplemented at its base by the 
juxtaposition of a piece of plane glass equal in thickness to the width of 
the base ; the prism itself is divided near its centre by a section parallel 
to the base ; it thus consists of three pieces, the whole shaped like a 
piece of the edge of bevelled glass. The apparatus is mounted in a 
brass cell, covered on both sides. The cover is pierced on each side by a 
central opening of 6 and 3 millimetres apertures respectively. By a 
simple, mechanism the three pieces of glass slide in the mounting, so that 
one or other of the two dividing lines with the .parts of the glass adjacent 
may thus be placed before the pupil of the seeing eye, — in other words, 
either the base of the prism and the piece of plane glass adjacent or the 
two pieces of the prism itself. Inasmuch as the two dividing lines and 
adjacent parts look exactly alike, monocular and binocular diplopia 
can be produced with the greatest ease and without the subject's know- 
ing, even if acquainted with the apparatus, what variety of diplopia 
(whether binocular or monocular) is present at any given instant. 



694 THE EYE AND NERVOUS SYSTEM 

The method of using the apparatus is simple. The eye that is 
alleged to be blind is lightly covered by the examiner's hand, and the 
subject asked to look at the test flame 2 or 3 metres distant and covered 
with the dark-red glass. The apparatus is then placed before the seeing 
eye, so as to bring the dividing line between the base of the prism and 
the piece of plane glass into coincidence with the horizontal diameter of 
the pupil. The patient sees two lights. The instrument is now re- 
moved and adjusted, without his knowledge, so as to bring the dividing 
line in the prism itself before the central aperture in the metal cell, 
and placed again before the seeing eye, the other eye being left uncov- 
ered, apparently and unintentionally. If the patient now sees double 
he is convicted, for the diplopia is binocular. If the simulator stub- 
bornly denies the existence of diplopia at first, both phases of the pro- 
cedure may be repeated in reversed succession. 122 

(4) Tests with Colored Glasses and Letters. — These are numerous. 
The one generally employed, or some modification of it, is known as 
Snellen's method. In general terms the patient is required to look at 
alternate red and green letters. The admittedly sound eye is now 
covered with a red glass, and if the green letters are read evidence of 
fraud is present. Instead of a red glass a green glass may be used, 
through which the red letters would be invisible. 

Very ingenious letters based upon the fact that red upon a white 
background viewed through a red glass disappears, and viewed through 
a green glass appears black, have been designed by Dr. von Hasel- 
berg. 123 I have found these very useful. 

(5) Tests with Stereoscopes. — Any of the ordinary stereoscopes may 
be utilized for this purpose and in the usual manner tests for binocular 
vision applied. Eor this purpose Worth's amblyoscope, and especially 
its modification by Black, should prove useful, on account of the ease 
with which it is adjusted to suit the position of the eyes and the condi- 
tion of the ocular muscles. Any refractive error may be previously elim- 
inated by adjusting suitable glasses, which are determined by the 
shadow-test. With this instrument full investigation of simultaneous 
vision of dissimilar objects, true fusion and the sense of perspective can 
be investigated, and its application to pretended blindness of one eye 
would be very easy. 

(6) Tests by Pseudoscopic Apparatus. — These depend upon the prop- 



12a Description taken from T. P. Schneideman's review of Baudry's paper, Oph- 
thalmic Review, xvii., 1898. 

123 Archiv f. Augenheilk., xlviii. 1901. 



LEUKOSES AND PSYCHOSES 695 

erties of a plane mirror, — that is, the malingerer is induced to read let- 
ters which he believes he is seeing with the admittedly sound eye, when 
really he is looking at them with the eye which he maintains is blind. 
An instrument of this character known as the box of Eles is much used 
abroad, and has consequently been employed in investigating the eyes of 
hysterical patients. It has been modified in many ways by a number of 
investigators. In our own country an ingenious test based upon this 
principle has been designed by Eridenberg, and is known as Fridenb erg's 
mirror test. 

Tests for Simulated Blindness of Both Eyes. — Naturally, it is 
much more difficult to detect a malingerer who claims to be blind in 
both eyes than one who maintains blindness of one eye alone. The 
tests which have been described are inapplicable, and formerly it used 
to be stated that the only satisfactory means of detecting the fraud 
was by placing a careful watch over the patient and taking him un- 
awares. The contraction of the pupil on exposure to light under such 
circumstances would not prove that there is sight in the eye, because, 
as Swanzy points out, a lesion of the centre of vision, or in the course 
of the fibres connecting this centre with the corpora quadrigemina, would 
produce absolute blindness, and would still permit an imperfect reaction 
of the pupil to light. 

Priestley Smith and Edward Jackson have suggested the following 
simple test for feigned binocular blindness : Place a lighted candle in 
front of the subject. Now hold a 6-degree prism base out before one 
eye. If both eyes see, the one behind the prism will move inward and 
on removing the prism will move outward, the other eye remaining 
fixed. 

Tests for Simulation of Changes in the Visual Field. — According 
to Koenig, no case has yet been published in which it has been proved 
that any one has succeeded in continuously deceiving an experienced 
physician by simulating concentric restriction of the visual field. Koe- 
nig' s experiments on this subject are very interesting. 124 He succeeded 
in simulating a concentric restriction of the visual field without fatigue 
phenomena to the extent of about 45 to 50 degrees on the temporal side, 
but when he attempted to simulate a larger visual field the irregularities 
became great. He was never able to simulate a visual field with only 
moderate constriction. Similar opinions are held by Wilbrand and 
Saenger. Simulation of simple concentric constriction of the visual 
field of high degree may be possible even without preliminary practice 
with the perimeter, but continuous deception would be difficult. 

124 Ueber Gesiclitsfeld-Ermiidung, 1893, p. 131. 



696 THE EYE AND NERVOUS SYSTEM 

Detection of simulated visual field phenomena could be secured by 
varying the examination with the campimeter and the perimeter, and 
especially by altering the point of fixation, as the malingerer would not 
likely be aware of the changes in the size of the visual field which must 
take place under these circumstances. In like manner, the tests with 
different sized test-objects would be sure to bring about detection. 
Baudry describes Schmidt-Rimpler's method as follows: While the 
test-object is situated on the limit of the field of vision of one eye, but 
is still seen by the patient, a prism that is too strong to be overcome is 
placed before this eye, both eyes being open. It is necessary to ascer- 
tain before the adjustment of the prism whether the test-object is seen 
by both eyes, or by one only. In the former case, if there has been 
simulation, the patient would have stated that he saw two objects, the 
true and the false image. If, on the contrary, he had been honest, he 
would have seen but one object. If originally the object is seen by one 
eye only, then if the patient is truthful, the prism placed before this 
eye makes the object disappear; the patient sees nothing. In case of 
simulation the object remains visible. 

Other methods of taking the field of vision will naturally suggest 
themselves. For example, Koenig's method in which, instead of examin- 
ing the individual meridians in the usual way, the examiner first picks 
out one and then another at random, thus rendering it difficult for the 
subject to remember the same point, as the point where the object enters 
the visual field. As already noted, von Reuss believes that his fatigue 
spirals, if present, are a real evidence of the absence of malingering, 
because they probably would be entirely unknown to the individual who 
was being examined. The capacity of the eye to recuperate has been 
recommended as a test for malingering. The principle of the method, 
according to Arnheim, is that an eye after nervous fatigue requires a 
greater time for its recovery than one that has become fatigued normally. 
Eor example, if a person whose visual field by diffused daylight ex- 
hibits a high degree of concentric narrowing very soon presents a normal 
field in a dark room, — that is, recovers rapidly,' — the original statement 
should be rejected as false. Simulation of the visual field is the sub- 
ject of a long research by Bichelonne. 125 

Simulation of partial achromatopsia and of dyschromatopsia can 
best be detected, according to Baudry, by means of Stillings' isochro- 
matic tables, or even better, by those of Pflueger. 

1Z5 Annales d'Oculistique, cxxix., 1903, p. 252. 



CHAPTEE XVII. 

MIGBAI^E, TETANUS, TETANY, EPILEPSY, BLINDNESS DUE 
TO UE^MIA AND GTHEE BLOOD STATES.* 

By JAMES HENDRIE LLOYD, A.M., M.D. 

MIGRAINE. 

Definition. — Migraine (Megrim, Hemicrania, or Sick-Headache) 
is a neurosis, characterized by pain in the fifth nerve, by affections of 
vision, and by gastric irritation. These three groups of symptoms may 
be said to constitute the tripod upon which the disease rests. In its 
simple or typical form they are always present, and they appear in a 
certain order of evolution. Moreover, migraine is a periodical affection, 
although its periods do not, as a rule, appear with regularity. Its onset 
is usually sudden; so sudden, in fact, that, like epilepsy, migraine is 
sometimes called one of the explosive neuroses. 

As the object of this article is to describe especially the ocular, or 
ophthalmic, symptoms of migraine, these symptoms will be given special 
prominence; but at the same time the other classical symptoms of the 
disease will be stated briefly in order that the account may not lack 
symmetry. 

Varieties of Migraine. — The disease has a variety of forms, some 
of which have been recognized only in recent years. It is significant 
that the two chief varieties are named after the ocular symptoms. First 
of these is the ordinary or classical migraine, which has already been 
defined, and which is now often called Ophthalmic migraine. In this 
form the affection of vision assumes special prominence ; with some 
well-marked variations in a few cases from the ordinary. Second, Oph- 
thalmoplegic migraine, in which a new and distinct element is present, 
— namely, a paralysis of the third nerve. Third, Psychical migraine, in 
which mental symptoms predominate in place of the usual ocular and 
neuralgic ones. In addition to these main varieties there are aberrent 
or abortive types, which, however, can best be described under one or 
other of the main groups. 

Causes. — Migraine has been known and studied for many years. 
In fact, it is one of the most familiar of nervous diseases, and there is 



* In the preparation of this chapter the author has to acknowledge tlu> valuable 
assistance of Dr. C. D. Camp, especially in the literary research. 

697 



698 THE EYE AND NERVOUS SYSTEM 

a very abundant literature upon it. 1 In spite of this fact, the essential 
causation and pathology of the disease are quite unknown, and much 
that is merely speculative is written about these aspects of it. 

Sex is probably not a determinative factor of great importance, 
although some authors, among whom is Eulenburg, contend that mi- 
graine is more common in women than in men. 2 But Sinkler has well 
remarked that this may be due to the fact that men do not seek treat- 
ment for the disease as persistently as do women. A far more important 
factor is age. In the great majority of cases the disease makes its 
appearance before or about puberty. 3 This, in truth, is only the expres- 
sion of another law, — namely, that migraine is usually an hereditary 
affection. The earlier the disease takes origin the worse and more 
inveterate it is. Cases in which the onset is delayed until after puberty 
are not, as a rule, so severe, and some authors have contended that 
migraine never begins in full adult life. But the statement by Tissot 
that the disease never begins after the age of 25 years may be open to 
question. Nevertheless, it is a rule that the longer it is delayed the less 
severe it is likely to be. 

Heredity is the most important factor in the causation of migraine. 
This law is manifest in a variety of ways. Eor instance, the heredity 
may be direct or by transformation. In the first mode, the transmission 
is from one or more migrainous ancestors, and may be shown in either 
a lineal or collateral relation. Thus the patient may acquire the disease 
from a parent or grandparent, in which case the descent is lineal; or 
it may appear collaterally, as in an aunt and nephew, or as in the case 
reported by Chabbert, 4 in which the disease showed itself in two sisters. 
The descent is said to be by transformation when the patient comes of a 
neurotic stock, in which other nervous diseases than migraine are prom- 
inent in the ancestry, as in Sieffer's case, 5 in which the disease appeared 
in two children of an insane mother and an alcoholic father. This 
method of inheritance by transformation is not uncommon in other 
nervous diseases, as in epilepsy, hysteria, and insanity. When the 
family stock is neurotic, a nervous disorder may, as it were, be ingrafted 

1 Liveing, On Megrim, Sick Headache, and Some Allied Disorders, London, 1873. 
Eulenburg, Ziemssen's Cyclopaedia, vol. xiv. p. 5. Sinkler, Migraine, in Pepper's 
System of Medicine, vol. v. p. 406. 

2 Eulenburg claims that 5 to 1 is the proportion of women to men ; a statement 
which could only be tested by elaborate statistics. 

3 The present writer, who is a victim of migraine, can recall attacks in the very 
earliest period of childhood to which his memory extends. 

4 Le Progres Med., 1895, p. 241. 

B Berl. klin. Woch., 1900, No. 30, p. 657. 



MIGKAHSTE 699 

upon it instead of being directly inherited. 6 Mobius claims that 90 
per cent, of the cases of migraine show heredity, and the disease has 
been known to descend through four generations, and to show itself in 
eight members of the same household. 7 Gowers s calls attention to, and 
gives instances of, the association of migraine with other nervous dis- 
eases in the same family, especially with epilepsy and pure neuralgia. 
Its relationship with gout has probably been exaggerated, especially by 
physicians who practice among an old and gouty population, and see 
gout in everything, and possibly have it themselves. Trousseau said 
that " migraine and gout are sisters ;" but the kinship is not easily 
recognized in America. 

There is more reason to suppose that migraine may be related to 
g astro-intestinal disorders. This is in part a popular notion, due to 
the gastric irritation which is one of the prominent symptoms in many 
cases, and which is not so much a cause as an effect of the disease. But 
there is some scientific ground for the belief : attacks may sometimes be 
aborted by prompt action on the intestinal tract; and the idea is in 
accord with the opinion that migraine is due to an autoinfection. In 
some cases as soon as vomiting occurs in the ordinary evolution of that 
symptom, relief begins, and may even be prompt. 

The action of overwork, overstrain, or overstudy may be contribu- 
tory to attacks by reducing strength, and impairing the resistive power 
of the system, but further than this it would not be safe or conservative 
to make statements. The same may be said of shock, moral and physi- 
cal, as well as of worry, grief, etc. 

Symptoms. — Migraine usually begins abruptly. It is, as we have 
said, one of the explosive neuroses. Some authors describe prodromes, 
such as a sense of depression or irritability for some hours or even a 
day beforehand. In rare cases a sense of elation or well-being precedes 
the attack. But in the commoner type of simple migraine the debut is 
unannounced. It comes in the midst of work or play, at any time of 
the day or night; in fact, it is sometimes nocturnal, occurring during 
sleep, the patient awaking in the midst of a full-blown seizure. 

The initial symptom, or aura, is usually sensory, and in the vast 
majority of cases it is visual. Exceptions, however, occur. A sensory 



6 Liveing (op. cit., p. 30) discusses Hereditary Transformations. The earliest 
discussion of this subject was by Moreau de Tours (De l'Etiologie de l'Epilepsie. 
1852). 

7 Oppenheim, Diseases of the Nervous System, translated by Mayer. 1000, 
p. 748. 

8 Diseases of the Nervous System, vol. ii. p. 837. 



700 THE EYE AND NERVOUS SYSTEM 

aura may occur in other parts of the body, as in a limb. The writer 
once had a patient who always felt a numb or tingling sensation in his 
arm. This mounted slowly upward, and as it reached the shoulder, 
neck, and head, it gradually disappeared, and the pain set in. The 
analogy with epilepsy is here striking, but an important difference is 
seen in the fact that the aura of migraine is of much longer duration 
than that of epilepsy. It is never instantaneous. It may, and usually 
does, endure for some minutes, even for a half hour. 

The patient, of course, from past experience, knows the full import 
of this aura. He usually drops everything and seeks a retreat, and not 
seldom he is much disquieted in mind. This mental state during the 
aura of migraine is important to recognize, for it is at this time in 
susceptible patients that hysteria may show itself and complicate the 
clinical picture. We shall refer to this subject later. 9 

The visual aura of magraine is characteristic, and has special sig- 
nificance for us in these pages. It is usually a form either of ambly- 
opia or amaurosis. The visual field is obscured in a variety of ways* 
This is its simplest type. The sight is confused, or partially obliterated. 
The outlines of objects seen are blurred and indistinct ; in other words, 
there is an amblyopia. The patient is not able to read, or reads with 
difficulty ; the same is true of objects and faces, which he can scarcely 
recognize. When a true amaurosis occurs, whole sections of the visual 
field may be obscured, and the patient may even be hemianopic. Thus 
there may be scotomata, in which large areas, as it were, are blotted out. 
There may also be scintillating lines, flashes of light and color, blazing 
or flaming splotches, and zig-zag or whirling figures, the forms of 
animals, terrifying apparitions, and other curious illusions. In 
mild cases of ordinary migraine these visual aurse may be but little 
marked, but in the type of the disease known as ophthalmic migraine 
they form an important element, and will be described more in detail 
under their appropriate head. 

It is the characteristic of the aura of migraine to disappear before 
the onset of the pain. This is the almost uniform rule. The visual 
field entirely clears, so that the patient, if he did not know otherwise 
from bitter experience, might suppose for a few minutes that he was 
entirely relieved of his attack. But the respite is brief; in fact, in 
some cases, the transition from the stage of visual defect to the stage of 
pain is almost imperceptible. 



9 Gilles de la Tourette (Traite de l'Hysterie, Paris, 1891) does not mention 
migraine among the exciting causes of hysteria, an omission which strikes the present 
writer as rather remarkable. 



MIGRAINE 701 

The pain of migraine is a neuralgia, usually intense, in the distri- 
bution of the fifth nerve. It is commonly described as a hemicrania 
(and in fact the word migraine is a French derivative of the Latin 
hemicrania), but it is not always strictly localized on one side of the 
head. It may be an intense frontal headache, and may even appear to 
radiate over a large part of the cranium. In other cases it is more dis- 
tinctly unilateral. Some patients even describe it as limited to one 
branch of the trigeminal, and the branch most involved is the first or 
ophthalmic division. 

This pain is cumulative, as Liveing has pointed out. It begins as 
a slight affection, but gradually increases, and in the course of an hour 
or two reaches its maximum. It is aggravated by motion, and by noise 
and light. The patient usually seeks absolute rest and seclusion. The 
character of the pain is described as boring, or throbbing: Du Bois 
Reymond, who was a victim of migraine, said that in his own case the 
pain throbbed in unison with the temporal artery. The intensity varies, 
but it is usually very great; in some cases, however, the pain is mild 
or even aborts early. Cases have even been described in which it is 
absent, or replaced by a vertigo ; these aberrant types will be referred 
to again. The duration of the pain also varies in different cases, 
although it is likely to be equal in the same case. As a rule, the pain 
does not endure over night; it is cured by a night's sleep. Cases in 
which it lasts for twenty-four hours are rare, although instances are 
reported in which it persisted for even two or three days. 10 The termi- 
nation of the pain is sometimes abrupt or critical ; this is true in cases 
in which it is relieved promptly by vomiting or by a copious movement 
of the bowels. It may be, and often is, terminated by sleep. 

Gastric disorder is very common in migraine ; in fact, it is one of 
the three characteristic symptoms. It is, as a rule, a late or terminal 
manifestation. Early in the attack, however, there is complete aversion 
to food, the patient not only feeling a physical disgust for it, but also 
apprehending its effect upon the nausea and vomiting which are almost 
sure to appear in time. That the nausea and vomiting are usually late 
or terminal symptoms, has long been recognized. 11 In some cases they 
have the character of a crisis, and the pain is often promptly relieved 
by emesis. This fact gives a hint for treatment. I have known a severe 
bout of migraine promptly aborted by the administration of an emetic. 12 

10 Piorry, Memoirs, etc. 

"Romberg, "A Manual of Nervous Diseases" (Syd. Soc. Trans., vol. i. p. 176). 

"An old writer, Robert Whytt (On Nervous Disorders, 1765, p. 305), fully 
recognized the value of emetics in these periodical headaches. " When they are 
regular as to their periods, vomits should be given an hour and a half beforehand." 



702 THE EYE AND NEKVOUS SYSTEM 

The same result can sometimes be obtained by a saline purge. In fact, 
the vomiting of migraine may be a natural mode of termination; a 
form of vis medicatrix naturae. It is nature's way of securing relief. 
In some severe cases, however, the vomiting is not always efficient at 
once, and it may recur during several hours before complete cure is 
effected. In some cases the stage of vomiting does not appear ; there 
may be very slight nausea or even none at all. This seems to be so 
especially of the attacks later in life, when the disease is losing its 
hold on the patient. But there are distinct aberrent types, even early 
in life, in which the stage of gastric irritation is conspicuous by its 
absence. 

The vomiting of migraine is often described as a cerebral vomiting, 
especially by the older writers, who affected to see in it an irritation of 
the pneumogastric nerve. It is true that it occurs without reference to 
the presence of food in the stomach, just as the vomiting of pregnancy, 
of sea-sickness, or of meningitis; and in severe cases may afford no 
relief. It is then attended with retching and great prostration, and 
instead of relieving, only adds to the patient's suffering. 13 

It is important to bear in mind that the varieties of migraine are 
not separable by hard and fast lines. They merge, as it were, into 
one another. Thus the distinction between " ordinary" migraine and 
ophthalmic migraine as made by some writers is an artificial one. In 
fact " ordinary" migraine is really only a form of the disease in which 
the visual aurae are merely not so conspicuous as in the ophthalmic 
variety. So of ophthalmoplegic migraine ; it is similar in many ways 
to the ordinary type except that it has in addition a motor disorder, 
— namely, a paralysis of one or other of the ocular nerves. In fact, 
it might perhaps be justifiable to say that there are two main types of 
migraine, a sensory and a motor type. In the one (ophthalmic) a 
sensory nerve, the optic, is especially involved. In the other (ophthal- 
moplegic) a motor nerve, the oculomotor, or rarely the abducens, is 
implicated. We must recall also that there is still another, but rare, 
type, in which the chief symptoms are psychical. These distinctions 
call to mind the analogy of migraine to epilepsy — an analogy which 
some authors are particularly fond of pointing out. 

OPHTHALMIC MIGRAINE. 

Originally the term ophthalmic migraine was used to bolster up a 
theory of the causation of the disease. This theory was that of Piorry, 
who held that migraine may be caused by overstrain of the iris and over- 

"Liveing, op. cit., p. 136 et infra. 



OPHTHALMIC MIGRAINE 703 

stimulation of the retina. Here we have a very early statement of the 
theory of eye-strain, which has since been worked up to such good pur- 
pose by some specialists. 14 The idea, in brief, was that an irritation of 
the iris and retina was the cause of the disease ; hence the theory itself, 
rather than the disease, should receive the name " ophthalmic." Piorry 
apparently had no knowledge of the motor, or ophthalmoplegic, form of 
migraine, and he was merely interested in establishing a plausible 
explanation of the disease. For him all migraine, however, was not 
ophthalmic. He recognized that it could be caused by other conditions 
than eye-strain. 

We speak of ophthalmic migraine to-day to distinguish that sensory 
form in which the visual auras are conspicuous, as distinct from the 
motor or ophthalmoplegic form in which there is paralysis of ocular 
muscles. The credit of first using the term " ophthalmic" for this 
purely sensory form of migraine is often given to Charcot (as are so 
many other things in neurology), but as constituting the commonest 
form of migraine, this variety had really been recognized for many 
years antedating the great French neurologist. 15 It is true, however, 
that since Charcot, and probably in part due to his influence, great 
pains have been taken by many writers to describe minutely the visual 
disorders of migraine, until now we can almost say that these disorders 
have been overdescribed. 

The visual symptoms in ophthalmic migraine may be divided as 
follows: 1. Amblyopia; 2. scintillations; 3. scotoma; 4. hallucina- 
tions. 16 

By amblyopia, as already said, is meant a simple blurring of the 
visual field. It is perhaps the commonest of these symptoms, and is 
often seen in simple cases of sick headache. It is not accompanied with 
distinct scotomata, or blotting out of definite areas of the field, but it 
is probably closely allied with them. In fact, it appears to be only a 



14 Piorry's work was his Memoire, etc. His theory is discussed in full by Live- 
ing, op. cit., p. 254 et infra. Piorry anticipated many of the specious arguments of 
later extremists, and, like them, he qu*oted cases to prove his position. By much 
the same process of logic it would be possible to prove that eye-strain is responsible 
for locomotor ataxia, or for cancer of the womb, or, in fact, for any disease which 
happens to appear in a patient who has a little astigmatism. 

15 Berbez, Les Migraines, in Gaz. Hebdom., 2 S. xxxvi. 19. The disease was 
recognized by Sazenne (1748), Plenk (1783), Stole (1795), Demours (1818). Wol- 
laston (1824) ; Madame de Pompadour was said to have been a victim. See also 
Miiller-Syer, Ueber Ophthalm. Migraine, in Berl. Klin. Woch., 1887, xxiv. p. 787. 

16 Galezowski (Etude de la Migraine Opthalmique, in Arch. Gen. de Med. 
Juin, 1878) gives a slightly different classification. 



704 THE EYE AND NERVOUS SYSTEM 

minor degree of amaurosis. In this amblyopia the patient sees objects, 
but sees them indistinctly; the outlines are confused, although with 
care and attention objects can be made out, as for instance, print or 
small articles. This would hardly be so if there were distinct scotomata, 
or localized blind areas. Still, it is difficult sharply to differentiate a 
mere amblyopia from areas of amaurosis or real blindness. Patients 
themselves are poor observers of these phenomena; they know that 
vision is obscured for a time, and they know the import of the symptom, 
but beyond that they do not attempt to observe or describe. An expert 
examination in these cases might possibly reveal some small spots of 




Fig. 1.— Scotoma scintillans. (After Charcot.) 

total blindness scattered over the field. But as the symptom is a tran- 
sient one, lasting only a few minutes, there is little chance to have these 
cases examined with care. In some cases this amblyopia passes into a 
stage of scintillation just before it ends. 

The scintillations of migraine are observed in many cases. They 
consist of waving or vibrating lines of light extending across or at the 
edges of the visual field. Not unfrequently they are confined to the 
edges of darkened areas, constituting then the phenomena known as 
scintillating scotomata. These spectral illusions were fully and graphi- 
cally described by Liveing. 17 They consist of a luminous border sur- 
rounding the blind area. This area, at first small, gradually expands, 
and as it increases in size, the scintillating rim makes its appearance 
and grows in intensity. Usually this rim is particolored (although not 



17 Op. cit., p. 75 et infra. Liveing gives very graphic colored diagrams of these 
scintillating scotomata. See the plate at the end of his work. 



OPHTHALMIC MIGKAIKE 705 

always so) and it is apt to assume a zig-zag shape, which was described 
by Fothergill 18 as consisting of luminous angles like those of a forti- 
fication. These images have been described in a variety of ways by 
different observers and they evidently vary somewhat in different 
cases. They are seen whether the eyes are open or shut, but 
sometimes better in twilight or darkness. Various colors may be inter- 
mingled with them, but in mild cases they are colorless, and not 
arranged in such fixed patterns as the imagination of some writers 
suggests. They may, in fact, be mere fleeting and dimly perceived 
vibrating lines, appearing just before the obscuring of vision passes 
away. In some cases the scintillation is centrifugal; that is to say, it 
appears at the edge of the shaded area, and tends to spread towards 
the circumference of the visual field, gradually dying out. In other, 
and rarer cases, the scintillation is centripetal, appearing at the edge 
and gradually extending towards the centre, thus covering the visual 
field. There has been some discussion whether the scintillations are 
binocular or monocular, but the weight of opinion is in favor of their 
appearing in both eyes, a fact which can be proved by the patient closing 
one eye at a time, when in some cases the scintillations disappear from 
the closed eye ; also as to whether they appear on only one-half of each 
field (hemiopic), and if so, on which half. We shall return to this 
subject in discussing the scotoma. 

Besides the wavy and zig-zag scintillations, there are other forms. 
These have been variously described as showers of sparks, colored hail 
or rain, flashes of light, flaming bodies, and spots of fire. These may 
either fill the whole field, or, what perhaps is more common, they appear 
around the edges of blind areas or scotomata. In fact, where there are 
genuine scotomata, these pyrotechnics could not well be seen except 
about the borders of the blind areas. It is possible to suppose that these 
various illuminations are due to an irritation of the optic fibres; 
whereas the scotoma, or blind spot, is due to a paralysis of optic fibres. 
The latter are conceivably the more central fibres, the former are the 
fibres arranged around the others whose functions are for the time 
abolished. 

These scintillations may be the only visual signs, except some slight 
amblyopia ; but in many cases they are accompaniments of scotomata or 
blind spots, to be presently described. In some cases they are not seen 
in the early years of migraine, but make their appearance later in life. 
Thus in the case of a woman aged 32 years, who had had migraine since 



Remarks on Sick Headache, Works, p. 597. 
45 



706 THE EYE AND KEKVOUS SYSTEM 

childhood, the muscse volitantes, colored spectra, flashes of light and 
scintillating scotomata, with photophobia, were only present after the 
thirtieth year. 19 

By a scotoma is meant a blind spot in the field of vision, and this 
spot is caused by and corresponds to an area in the retina in which the 
function of sight is abolished. In migraine such scotomata are not 
uncommon as part of the visual disorder present in the early stage; 
in other words, as part of the aura. It is from this symptom that 
migraine is often called " blind-headache." These scotomata may be 
variously placed and of variable extent. Thus they may be central, or 
near the centre, or, as is not unusual, spread over a part of one side of 
the field. They are, as a rule, present in both eyes. The most important 
is the scotoma scintillans, already referred to, in which the edge of the 
blind area is the seat of zig-zag or " fortification" lines of wavy and 
particolored light. 

The effect of these scotomata is to cause curious impairment of 
vision. Objects are seen only in part. Thus Mobius 20 had a patient 
who said that men walked about without their heads ; and Abernethy, 
who was a victim of migraine, said humorously that he could see no 
more of his own name than the " knee" (ne) and the " thigh" (thy). 21 
The scotoma lasts but a short time, ten to twenty minutes, rarely as 
long as an hour; although Galezowski recorded an unusual case in 
which it lasted for five months, without objective findings. In fact, 
ophthalmoscopic examination during the time of the attack reveals 
nothing. 

This is the uniform experience. These attacks are sometimes at- 
tended with intense photophobia. In some cases, as Mobius points out, 
the lids droop, but this may be due to an instinctive desire to shut out 
the light. He also says that pupillary changes sometimes accompany 
attacks of ophthalmic migraine, but in this he is not confirmed by many 
observers. They are seen, however, in the ophthalmoplegic form. 

These scotomata may be so extensive in some cases as to include one- 



19 Da Costa, "A Case of Ophthalmic Migraine," in the Jour. Nerv. and Ment. 
Dis., 1890, xv. p. 213. 

20 Migraine, in Nothnagel's Spec. Path. u. Therap., Bd. xii. See also Dianoux, 
Scotoma Scintillant ou Amaurose Partielle Temporaire, These, 1875. 

21 This must have been a stock joke of Abernethy's, and made to do frequent 
duty, or else its author has been much misquoted. Abernethy perpetrated this pun 
in a lecture, published in the Lancet, October 21, 1826, and said the disorder of 
vision in his own case was caused by an injury (a blow on the nose), and he made 
no reference whatever to migraine. The lecture has recently been republished in the 
Lancet (October 22, 1904, p. 1170). 






OPHTHALMIC MIGKAIKE 707 

half of each visual field, in which case there is an hemianopsia. This 
has been reported by not a few observers. 22 When these scotomata are 
thus hemianopic they may appear either on the side upon which the 
pain is present or on the opposite side. When the pain is strictly local- 
ized on one side, it is usually on the side of the hemianopsia. This is 
true also of other sensory aurse, such as a numb feeling in a limb; it 
is usually on the same side as the pain. Exceptions, however, occur, 
as was pointed out by Liveing, Calmeil and others. This subject 
needs more careful study. The hemianopsia may be lateral or ver- 
tical. 23 

Visual hallucinations have been observed in migraine. But they 
must be rare, for they are reported by only a few observers. S. Weir 
Mitchell 24 has put such cases on record. Thus a school-teacher, aged 
30 years, saw a large, black and hairy dog which disappeared as the 
pain began. Another patient saw an image of her dead sister. Still 
another saw an image of a near relative covered with blood, especially 
if the attack happened at her menstrual period. In a fourth case there 
was a scotoma scintillans, but after some attacks the patient had visions 
of a trellis of silver covered with vines and flowers of brilliant hue, best 
seen with the eyes open. Some of these were associated with geometrical 
figures, pink or red. It would be interesting to know, in such cases, 
whether there were hysterical symptoms present, or whether such com- 
plex visions might be the result of suggestion. Certainly they are not 
of the ordinary type of visual phenomena seen in migraine, especially 
when they occur after the attack. The analogy of such cases with 
epilepsy is also suggested, but of course not proved; for visual hallu- 
cinations have been noted among the rare aurse of the sacred disease. 
A curious form of visual hallucination, or more properly illusion, has 
been noted in migraine by Gowers. 25 This might be described as a 
projection outward of the scintillation or coruscation ; thus to one 
patient it appeared as though a plate which sat before him on a table 



22 Mobius, op. cit. Galezowski, op. cit. Da Costa, op. cit. Dufour. Sur la 
vision nulle dans l'hemiopie, in Rev. Med. de la Suisse r'oun., 1889, p. 445. Schmidt's 
Jahrb., ccxxvi. p. 74. Liveing, op. cit. Airy, On a Distant Form of Transient 
Hemiopia, in Philosoph. Trans, for 1S70, p. 247. Harris. Eemiopia, etc.. in 
Brain, 1897, vol. xx. p. 308. 

23 Airy made use of the word " teichopsia" (from ra^oc, a wall, and one vision) 
for this symptom, especially where there is the appearance of a " fortification" line. 

24 Neuralgic Headache, with Apparitions of Unusual Character, in Am. Jour. 
of the Med. Sci., vol. xciv. p. 415. 

23 Diseases of the Nervous System, vol. ii. p. 846. 



708 THE EYE AKD KEKVOUS SYSTEM 

was surrounded with the colored spectrum. But neither Gowers nor 
Liveing mentions true hallucinations. 26 

A few observers have reported cases of that rare symptom known as 
" hemianopic hallucinations." Harris 27 has collected a few references 
to these cases, from which it is evident that they are due to organic 
lesions chiefly. Tuke 28 mentions them in connection with migraine 
and epilepsy, but Harris gives no instance that was not due to organic 
disease. The latter author indulges in some speculation about the 
origin and seat of such visual symptoms, and adopts Hughlings- Jack- 
son's phraseology. For instance, he holds that a visual aura in migraine 
is caused by an " epileptic discharge" — a needless confusion of the 
two diseases. In hemianopic hallucinations the patient sees the false 
images with one-half of the visual fields only. 29 

In rare cases migraine is associated with symptoms of other affec- 
tions. Among these is aphasia. Charcot 30 made the distinction be- 
tween simple ophthalmic migraine in which the symptoms are scotoma 
scintillans, transient hemiopia, headache, and vomiting; and migraine 
ophthalmique accompagnee, in which in addition there are a more or 
less permanent hemianopsia, difficulty in speech, and numbness of one 
side of the face. He claimed that both the prognosis and treatment 
are different in this latter and graver type. But this distinction was 
not original with Charcot, for it had been made long before him by 
Lebert' 31 and others. 32 It is doubtful, however, whether all of the re- 
ported cases w 7 ere true instances of migraine. Some of them appear to 
have been due to cerebral disturbance, probably vascular or toxic, in 
which there was not only aphasia but also transient hemiplegia and 
hemiansesthesia, with mental confusion; and the suspicion is aroused 
that these attacks in some cases may have been obscure symptoms of 
Bright's disease, or even of hysteria. Fere 33 also draws attention to 



26 Zacher (Berlin, klin. Woch., xxix. p. 694) mentions a case in which hallucina- 
tions of sight were followed by epileptic attacks, but the case was evidently one 
of epilepsy with a visual aura, not migraine. 

'* Hemianopia, in Brain, 20, 1897, pp. 318, 360, 361. 

- 8 Diet. Psych. Med., vol. i. p. 568. 

29 Liveing, op. cit., who described the hemiopia of migraine very fully, apparently 
makes no mention of hemianopic hallucinations, except the fortification spectra 
already alluded to in the text. 

""Lecons de Mardi, 1887, p. 27. 

31 Traite Prat, des Mai. Cancereuses, Paris, 1851. 

83 Liveing discusses the subject at length in his classical work. 

33 Sur la Migraine Ophthalmique, in Rev. de Med., 1883, iii. p. 194. 



OPHTHALMIC MIGRAINE 709 

the fact that these cases may mean a grave prognosis. He reports a 
case in a patient, 53 years old, in whom the speech defects became more 
frequent. There were finally permanent aphasia, right hemiplegia of 
the face and arm, local convulsions becoming general, and death. There 
seems to be no sufficient reason to attribute all these grave cerebral 
symptoms to migraine, simply because they occurred in a migrainous 
patient. Meige 34 also reports a case, in which there appears to have 
been disease of the cerebral arteries. 

Some authors have also spoken of migraine as an early symptom of 
general paresis. It is doubtful what the exact relationship is, even if 
there be any relationship other than a coincidence. 35 Blocq thinks that 
the relation is anatomical. He reports three cases, all with neurotic 
heredity. There is no reason, of course, why a patient with migraine 
should not also develop general paresis, but a distinct pathological rela- 
tionship seems not to have been made out. Mickle says that precedent 
migraine often disappears at the development of general paresis ; and 
Chase 36 observes that severe headache is sometimes seen as an early 
symptom of paresis, but he does not call such headache migraine. There 
is possibly some confusion of identity in some of the reported cases. 

The same may be said of locomotor ataxia, of which migraine is 
sometimes described as a prodromal affection. 37 This is certainly not 
observed in the vast majority of cases of tabes, and the association is 
probably accidental. 

That some of the bizarre symptoms noted above are due to hysteria, 
there can not be much doubt. Among these are speech-defects, hemi- 
anesthesia, hemiplegia, convulsions, and other hysterical stigmata. This 
truth has been fully recognized by some authors, but strangely over- 
looked by others. In fact, we have in migraine an exciting cause of 
hysteria. This simple fact is readily understood in view of the alarm- 
ing nature of the migrainous symptoms, such as the vivid scintillations 
and blind spots, followed by a period of distressing headache and vom- 
iting. More or less mental or emotional disturbance is not uncommon 
in the first stage of migraine. The patient is usually, at least, depressed, 
and sometimes alarmed. He knows full well the import of the visual 
aura, and understands that he is fated to a painful bout, which will 



a4 Rev. Neurologique, Xo. 18, 1904. 

33 Parinaud, Migraine Ophthalmique au debut de la Paralysio Generate, in 
Arch, de Neurolog., 1883, v. p. 57. Blocq, Migraine Ophthalmique et Paralysie 
Generate, in Arch, de Neurolog., xviii. p. 321. 

36 General Paresis, 1902, p. 146. 

37 Raullet, Etude de la Migraine Ophthalmique, These de Paris. 1883. 



710 THE EYE AKD NERVOUS SYSTEM 

knock him out for a day or two, and entail much physical suffering. It 
is small cause for wonder, therefore, that in some neurotic patients the 
symptoms of hysteria should complicate the case. The writer has known 
such cases, and has been led to suppose that they are more common in 
children and young persons. Babinski 38 reports such a case in a girl 
six years old. She had such hysterical symptoms as hemianesthesia, 
dyschromatopsia, contraction of the visual fields, and globus; these 
were associated with, or excited by, the migraine, especially the scotoma 
scintillans and the violent hemicrania. To show how susceptible such 
patients may be to suggestion, it was only needful to make pressure on 
the sixth dorsal vertebra (in this case, a hysterogenous point) to cause 
the appearance of a scotoma. In some cases in which so-called epileptic 
equivalents have been reported, the convulsions were probably hysteri- 
cal. 39 In cases in which the scotoma is reported as unduly prolonged 
(as in Galezowski's case, referred to on a preceding page) it is likely 
that the symptom is due to the great neurosis. 

Reference has already been made to the forced resemblance between 
migraine and epilepsy. Both diseases are explosive, both are preceded 
by an aura, and both occur in neurotic patients, but beyond these few 
points the resemblance ceases, or at least is not marked. 40 An important 
question arises, however, as to the possibility of the transition from 
migraine to epilepsy. This claim is made by some authors, but the 
present writer has never seen or heard of an authentic case, and does not 
believe in the doctrine. The cases reported are probably instances of 
hysterical convulsions, or they are cases in which migraine and epilepsy 
coexist in the same patient — which is by no means a proof that the one 
disease causes or merges into the other. 41 

Pathology. — It is proper here to make a brief reference to the claim 
that migraine is caused by eye-strain. As already said (page 703), this 

38 De la Migraine Ophthalmique Hysterique, in Arch, de Neurolog., November, 
1890, p. 305. In this paper Babinski reports several cases of hysteria caused by 
migraine. Among the hysterical stigmata were mutism, hemianesthesia, anaesthesia 
of the palate, monocular diplopia, and contraction of the visual fields. He even 
claimed that attacks had been cured with hypnotism. Thomas (Migraine Oph- 
thalmique Associee de Nature Hysterique chez l'enfant, in Rev. Med. de la Suisse, 
xii. p. 800) reports the case of a boy whose migrainous attacks were accompanied 
with paresis of the right side and difficulty in speech, lasting an hour, with other 
stigmata of hysteria. See also Fink, Des Rapports de la Migraine Ophthalmique 
avec l'Hysterie, Paris, 1891. 

89 Mengazzini, Rev. Sper. di Freniatria, abstracted in Cent. f. Nervenheil., xvii. 
p. 162. 

40 Mobius, Ueber Migrane, in Cent, f, Neurolog., viii. p. 244. 

41 Spiller, The Relation of Migraine to Epilepsy, in Am. Journ. Med. Sci., 1900. 



OPHTHALMIC MIGKAHSTE 711 

theory was advanced by Piorry many years ago, but it fell into obscurity 
until revived recently by a few extremists. Martin has reported no 
fewer than 352 cases in which he thought he was able to detect a low 
degree of astigmatism as the cause of migraine, 42 but he probably mis- 
took a post hoc for a propter hoc, sl conceivable error in a hasty observer 
in view of the wide prevalence of astigmatism. Gould, 43 who has writ- 
ten voluminously on the subject, makes the sweeping claim that the near 
use of astigmatic eyes is always required to produce migraine, but his 
conclusions are widely rejected. Paul Bert 44 held that scintillating 
scotoma, generally attributed to fatigue of the retina, is in reality due 
to fatigue of the nervous centres ; but there seems to be no real scientific 
warrant for such dogmatic statements. 

Attempts to describe the pathology of migraine have been equally 
speculative and futile. Dufour 45 held that the disease, or rather the 
scotoma, was due to " vasomotor anaemia" of the occipital region of the 
brain. Ball 46 supposed it was due to a " local asphyxia." Latham 47 
believed that the visual disturbance was due to " excitation of the 
sympathetic," and so on. Recently the tendency has been to suppose 
migraine to be due to an infection ; but this theory is merely an evi- 
dence of the present trend towards a humoral pathology, and it cannot 
be said to rest upon solid facts. 

German writers, as Eulenberg, 48 have been fond of insisting on the 
distinction between the " angiospastic" and " angioparalytic" types of 
migraine. The distinction is based on the supposed condition of the 

42 Ann. d'Oc, xeix. pp. 24 and 205. 

43 Journ. Am. Med. Asso., Jan. 1904. 

44 Comp. Rend, des Seances de la Soc. de Biol., 1882, p. 571. The very extrava- 
gance of the claims of some of these writers tends to cause distrust of their opinions. 

45 Sur la Vision Mille dans l'Hemiopie, in Rev. Med. de la Suisse, 1889, p. 445. 

46 Quoted by Raullet, Etude de la Migraine Ophthalmique, These de Paris, 
1883. 

47 On Nervous or Sick Headache, 1873. 

48 Op. cit. Quaglino (Annali del Ottamologia, 1871, p. 7, cited by Raullet) held 
that ophthalmic migraine was due to " spasm of the arteries of the retina." It is 
extraordinary how completely the advocates of these vasomotor theories ignored 
the question of a possible cause for the alleged action of the vasomotors. There 
could hardly be a vasomotor spasm without something to cause it. These theories 
grew out of the solidist school of pathologists, who explained every disease by a 
change in the tissues, and complacently ignored the necessity for finding causes for 
such alleged changes. 

Among other works on ophthalmic migraine are the following: Robiolis, Con- 
tribution a l'Etude de la Migraine, etc., These de Montpellier. 18S4. Baralt, Con- 
tribution a l'Etude du Scotome Scintillant, etc., These de Paris, 18S0. Dianoux, 
Du Scotome Scintillant, These de Paris, 1875. See also Brewster, Philosophical 
Magazine, 1865, p. 503. 



712 THE EYE AND NEKVOUS SYSTEM 

vasomotor supply. In one form the face is pale, the eye hollow, the 
pupil dilated, and the arteries tense. Eulenberg even claimed to have 
found the temperature of the external ear reduced four-tenths of a 
degree ! In the other form the reverse of all these conditions is seen, 
and the temperature of the ear rises two-tenths of a degree. These fine 
physiological studies have never thrown the slightest light on the nature 
of migraine, for the question still remains, What causes the vasomotor 
change ? 

Some pathologists reach impotent conclusions about migraine, as, 
for instance, Oppenheim, who, at the autopsy of an old migrainous 
patient, found central softening from embolism as the cause of death, 
and who thought vasomotor disturbance had caused the old migraine 
and likewise the recent embolus. Surely this is a far-fetched conclusion. 

OPHTHALMOPLEGIC MIGRAINE. 

This form of migraine is characterized, as its name implies, by a 
paralysis of some of the ocular muscles. This paralysis is usually a 
sequel or result of the migrainous attack, and is, of course, like that 
attack, periodical. The muscles involved are, as a rule, those supplied 
by the third nerve, but a few cases have been observed in which the 
paralysis has occurred in the fourth or the sixth nerve. 

The term " ophthalmoplegic migraine" was suggested by Charcot 49 
in 1890, but Charcot was not the first to record an instance of this 
interesting disease. Mobius 50 is usually credited with this distinction 
(in 1884), but even before that date Adams, 51 in 1877, had reported 
a case of paralysis of the left third nerve with remarkable intermission, 
relapse, and recovery, which may have been an instance of ophthalmo- 
plegic migraine. Saunby, 52 in 1882, had reported a clear case of 
migraine with paralysis of the third nerve, and the distinction of prior- 
ity seems indubitably to belong to him. After 1884 reports of cases 
became quite numerous; and merely to repeat the titles of the papers 
would now be a task quite irksome and prolonged. 53 

49 Sur iin cas de Migraine Ophthalmoplegique, in Cliniques des Maladies du 
Syst. Nerv., 1892, p. 70. 

50 Neurol. Beitrag., iv. Deut. Zeit. f. Nervenheilk., Bd. xvii. 299. 

51 Ophthalmic Hosp. Rep., Dec, 1877. 

52 Lancet, 1882, Sept. 2, p. 345. 

53 The following are the principal early reports besides those already named: 
Snell, Lancet, 1885, vol. ii. p. 939. Clark, ibid. Beevor, ibid. Thompson, Char. 
Ann., 1885, p. 567. Manz, Berl. klin. Woch., 1885, Nr. 40. Parinaud, Soc. d'Oph. 
Francaise, Janv. 29, 1895; also Schmidt's Jahrb., Bd. ccvii. p. 245. Suckling, Brain, 
1887, July, p. 241. Senator, Zeit. f. klin. Med., Bd. xiii. p. 252. See Charcot's 
paper, op. cit., for a brief discussion of the history, with references to early cases. 



OPHTHALMOPLEGIC MIGKAIKE 713 

In ophthalmoplegic migraine we have that motor' type of the dis- 
ease, to which reference has already been made, in distinction from the 
sensory or ophthalmic type, which has just been described. This dis- 
tinction seems warranted by the fact, as pointed out by Charcot and 
others, that the ophthalmic aura, such as the scintillating scotoma, is 
often absent in this motor form, whereas the paralysis of some of the 
ocular muscles is pronounced and characteristic. 

Ophthalmoplegic migraine generally pursues about the following- 
course: The attack is usually divided into two periods. First, the 
period of pain. Second, the period of paralysis. 




Fig. 2.— Paralysis of the right third nerve, to illustrate ophthalmoplegic migraine, although in this 
case the disease was brain syphilis. 

The period of pain begins brusquely, and the pain itself is localized 
upon one side. This localization is more marked than in ordinary 
migraine. The pain is often centred about the eye, — i.e., in the oph- 
thalmic division of the fifth nerve. The visual aura, such as scotoma 
scintillans, hemianopsia, etc., is usually absent. 54 Although the pain 
begins suddenly, it does not reach its maximum at once, but gradu- 
ally increases in severity; and although it is usually localized, it may 



"Charcot, op. cit. Suckling, op. cit. Kollarits, Deut. Zeit. f. Nervenheilk.. 
1904, Bd. xxvi. H. 1, 2. 



Y14 THE EYE AND NERVOUS SYSTEM 

in some cases extend even to the occiput. But it almost invariably 
remains upon one side. This pain may become most severe. Its dura- 
tion varies in different cases; in some it is short, a matter of a few 
hours; in others long, extending over some days. As in ordinary 
migraine the bout of pain is usually terminated by vomiting. This 
vomiting may be in the nature of a crisis ; it appears to bring relief, 
and this relief is prompt, even sudden. In other words, the termination 
of the painful period is as abrupt as its beginning. 

As the pain and vomiting disappear the second period, or period of 
paralysis, asserts itself. This paralysis has certain characteristics, which 
are quite common in the majority of cases, although exceptions to the 
rule occur, as will be noted. The palsy is an ophthalmoplegia, and in 
the great majority of cases it is confined to the third nerve. The paraly- 
sis of the third nerve is usually total and complete. That is to say, it 
is total in the sense that all the branches of the nerve are involved, and 
it is complete in the sense that the muscles are not merely weakened but 
absolutely paralyzed. Hence we have presented the classical picture 
of a third-nerve palsy, such as occurs from an organic lesion. • The 
upper eyelid droops, the eyeball is rolled outwards, and the pupil is 
dilated. Moreover, the pupil is quite immobile; it does not react to 
light, nor upon convergence or accommodation. Thus there is an 
ophthalmoplegia both externa and interna. 

The duration of these two stages varies, as we have said, in different 
cases. The period of pain is usually shorter than the period of paraly- 
sis. This paralysis, indeed, may last for a comparatively long time. 
In Schilling's case 55 it endured for from four to six weeks ; in Pari- 
naud's case for from two to three months. But in many cases the 
paralysis disappears in a few days. 56 

Charcot claimed that the paralysis is always confined to one eye; 
it never shifts from one eye to the other, and in a given case it always 
appears in the same eye in succeeding attacks. It may be in the left 
eye or in the right eye, but always in the same eye in the same patient. 
But this rule has been found by other observers not to be constant. Thus 
Demicheri 57 observed a case in a woman aged 64 years in which the 
palsy appeared first on the left and then on the right side; and 
Karplus 58 had a case in a woman aged 39 years in which there was 

55 Munch, med. Woch., 1903, No. 18, p. 776. See also Wilbrand and Saenger ; 
Die Neurologie des Auges, 1, 2 Ab., p. 575. 

56 Manz, op. cit.; Borthen, Klin. Monatsblat. f. Augenheilk., Sept. 1893. 
57 La Clinique Ophthalm., 1899, 18. 
68 Jahr. f. Psych., xii. p. 158. 



OPHTHALMOPLEGIC MIGKAIKE 715 

a left-sided oculomotor paralysis following migraine, Avhich reappeared 
on the right side. These cases, however, appear to be quite exceptional. 

Some cases of this recurrent palsy of the oculo-motor nerve observe 
a distinct periodicity, but this is not true of all. Kollarits's patient had 
attacks three or four times a year. In Suckling's case they occurred 
every two weeks ■ in one of Karplus's patients every year. In women 
the attacks have occasionally been seen to coincide with the menses. 
On the other hand the occurrence has sometimes been most irregular 
and at very long intervals. In one case 59 the onset was in the patient's 
thirteenth year, and the attack was very brief (only one day) ; then 
after an interval of ten years the disease recurred, and later became 
periodical at short intervals — two or three attacks monthly. Charcot 
called special attention to the fact that the duration of the access of 
ophthalmoplegic migraine, even the shortest, is usually longer than the 
access in ordinary migraine; and he thought it was the rule that in 
cases in which the attacks ivere short the intervals between the attacks 
were also short. When the attack does not exceed four or six days, the 
seizures may occur as often as once a month, as in Senator's case. 

The onset of migraine in these cases is often at a very early age ; 
in fact, the majority of them seem to date their origin from childhood 
or even from infancy. Thus in one case 60 the first attack happened 
when the patient was only a year old; the second attack occurred at 
seven, and thereafter the attacks recurred every nine or ten months. In 
Suckling's case the attacks had occurred also since infancy; and de 
Schweinitz 61 has reported a case which began in an infant one year 
and a half old, and which, after repeated attacks, left a permanent 
paralysis of the third nerve. !Rot a few of the cases began before or at 
the age of puberty; thus cases are recorded which had their onset at 
5J, 7, 8, 11, 13, and 14 years. 

It is a noteworthy fact that there is often a change of type in these 
cases. Thus in the early years the disease presents the type of an 
ordinary or ophthalmic migraine, and this changes later to the ophthal- 
moplegic type. This seems to prove that the latter form is a genuine 
migraine, and not a different disease, as some observers have claimed. 
This fact, however, does not avoid the inference that some few excep- 
tional cases of recurrent palsy of the third nerve are not cases of true 

59 Darkschewitsch, Arch. f. klin. Med., Bd. xlix. S. 457. 

60 Ormerod and Holmes Spicer, A Case of Recurrent Paralysis of the Third 
Nerve, with Migraine, in Lancet, Dec. 21, 1895, p. 1580. 

61 Recurrent Oculomotor Palsy, with a Case, in Boston Med. and Surg. Journ., 
1895, p. 341. 



716 THE EYE AND NERVOUS SYSTEM 

migraine, a fact which will be discussed under the head of diagnosis. 
In cases, however, in which there is this change of type the correlated 
facts are too obvious to admit of a doubt that the two types are really 
the expression of one and the same morbid entity. Chabbert 62 reports 
the case of two sisters who had ophthalmic migraine with scotoma scin- 
tillans, hemianopsia, etc. In one of these cases at 52 years the attacks 
became complicated with right-sided oculomotor palsy. Joachim 63 had 
a patient who had ordinary migraine from the eleventh year ; at twenty 
years of age she suddenly developed oculomotor palsy with diplopia, etc., 
after a rather slight attack. Somewhat similar cases have been reported 
by Stezeininski, 64 Bouchaud, 65 Stock, 66 Senator, 67 and Lapersonne. 68 
In Bouchaud's case the oculomotor palsy developed in a woman aged 
sixty years who earlier in life had had migraine. Charcot 69 records a 
case in which ordinary migraine had existed in early life and had ceased 
in the patient's twenty-third year ; but in her thirtieth year she began 
with attacks of recurring oculomotor palsy. It is impossible, as Char- 
cot says, not to see some relationship between these two types, even 
widely separated as they were in time. 

It has been observed in some cases that after repeated attacks of 
ophthalmoplegic migraine the paralysis tends to become more and more 
persistent and even in time to become permanent. This is true espe- 
cially of those cases in which the period of paralysis is prolonged and 
occurs at prolonged intervals. In such cases the intervals between the 
attacks are not always entirely free from the appearance of some paresis ; 
and even the pain may persist in slight degree. Senator, noting these 
facts, said that the disease was not so much periodical as it was " con- 
tinuous with periodical exacerbations." The suggestion of an organic 
lesion, such as an inflammation of the nerve-trunk, is likely to occur to 
the observer of these cases ; and in fact some such organic lesion may 
be the cause of the permanent palsy. In Schilling's case there was some 
interparoxysmal paresis; as also in Ballet's case. 70 In a case recorded 
by Karplus 71 a permanent ptosis was left ; an interesting instance of 

02 Le Progres Med., 1895, No. 15, p. 241. 
63 Deut. Arch. f. klin. Med., Bd. xliv. p. 185. 

64 Pec. d'Ophthalmol., September, 1897. 

65 Presse Med., Avril 28, 1897. 

66 Cited by Paderstein, loc. cit. 

67 Loc. cit. 

os p rogr g s Med., 1903, No. 10, p. 161. 
09 Op. cit. 

70 De la Migraine Ophthalmoplegique, These de Paris, 1896. 

71 Jahr. f. Psych., vol. xii. p. 158. 






OPHTHALMOPLEGIC MIGKAIKE 717 

only one branch of the third nerve being permanently injured. In de 
Schweinitz's case in a child a divergent squint remained in the intervals, 
but the ptosis appeared and disappeared with each succeeding attack 
until the twenty-eighth year, when the ptosis too became permanent. In 
Ormerod and Spicer's case some of the paralyzed muscles had never 
recovered. Whatever may be the explanation of this permanent palsy 
in a disease which is usually called " functional," it is necessary for the 
present to hold in abeyance any theory which does not include the 
possibility of an organic lesion. It is likely indeed that this permanent 
injury to the motor nerves is the result of some irritant, such as a toxin, 
acting as the essential cause of migraine. 

The paralysis in ophthalmoplegic migraine is not always limited to 
the third nerve, and in a few rare cases the third nerve has escaped 
entirely, while the fourth or the sixth nerve has been implicated. Leiz- 
enberger 72 reported the case of a man aged 29 years who had 
migrainous attacks every eight days followed by isolated paralysis of 
the trochlear nerve. Ordinarily when the fourth nerve is involved, it 
is in association with the third nerve, as in the case recorded by Cou- 
touzis, 73 in which there was conjoint palsy of the oculomotor and 
trochlear nerves with violent headache. The attacks were distinctly 
migrainous, with pain in the parietal region, vomiting and salivation 
for forty-eight hours, followed by the loss of power in the two nerves. 
There was no scotoma or hemianopsia. The paralysis lasted for twelve 
days, but longer in the superior rectus muscle. 

Paralysis of the sixth nerve has been observed in a small number 
of cases. Charcot has made such a case the subject of a detailed report. 74 
The patient, aged 41 years, had ordinary migraine beginning at the 
age of 16, which ceased at 32 years, to be followed at 38 years by a 
violent attack of left-sided neuralgia without nausea or vomiting, and 
lasting for four hours. Diplopia resulted, and was due to an isolated 
paralysis of the abducens nerve, lasting for five days. After an interval 
of two years a similar attack, but on the right side, occurred, and was 
followed by paralysis of the oculo-motor nerve ; six months later oc- 
curred still another attack, but on the left side, followed by paralysis 
again of the sixth nerve. Charcot was obliged to conclude from this 
case that the attacks are not always unilateral ; the fact was shown, how- 
ever, that the third and sixth nerves, although paralyzed in opposite 
eyes, were paralyzed each on the same side in each attack. This palsy in 

72 Abstracted in Nenr. Cent., xvii., 2, p. 73. 
73 Journ. de Med. et de Chir. Prat., Avril 25, 1897. 
74 Rev. Neurolog., 5, 8. 



718 THE EYE AND KEKVOUS SYSTEM 

Charcot's case was long continued, but not in all branches equally, for 
the levator palpebrae and superior rectus were most, or longest, involved. 
Paderstein 75 reported a case in which there was a paralysis of the left 
abducens nerve and a weakness of the left superior rectus muscle, with 
equal pupils and no paresis of the levator palpebrse; showing again 
that the various branches of the third nerve are not always equally at 
fault. De Schweinitz 76 also put on record a case of recurrent palsy of 
the abducens nerve, which began when the patient was only one year 
old, and recurred one year later, and again in five years. The attack 
was ughered in with fever, nausea, vomiting and headache, and recovery 
after each attack was comj)lete. 

A very rare case was that reported by Rossolimo, 77 in which a recur- 
rent paralysis of the seventh nerve was always preceded by a migrainous 
attack. The paralysis was in the superior as well as the inferior dis- 
tribution of the nerve; so it was evidently peripheral. There was no 
involvement of ocular muscles. Rossolimo referred to a similar case 
reported by Hatschek. 78 

It is a remarkable fact that but few authors make mention of anaes- 
thesia of the fifth nerve in these cases of ophthalmoplegic migraine. 
The inference is that such anaesthesia seldom occurs. When we con- 
sider the fact that the nerve-storm vents a large part of its fury on the 
fifth nerve, causing the intense headache, and that the cause, whatever 
it is, is sufficient to paralyze motor nerves, it seems rather remarkable 
that the sensory nerve is not also paralyzed. Knapp 79 has reported a 
case in a man aged 41 years in which there was a distinct anaesthesia in 
the first and second divisions of the fifth nerve. The attack was of long 
duration, and the sensory condition was what is known as anaesthesia 
dolorosa ; that is, there was a partial loss of sensation, the face feeling 
as though it were covered with leather, and objects were felt as through 
this substance; associated with pain on pressure or even on slight 
touch. Haynes 80 also has reported a case in which the eye felt sore, 
and there was anaesthesia of the supra-orbital branch of the fifth nerve, 
accompanied with or followed by ptosis. 81 

73 Deut. Zeit. f. Nervenheilk., xv., 5 u. 6, p. 418. 

7G Philada. Polyclinic, vi. 39. 

77 Neur. CentbL, 1901, S. 744. 

78 1894, Klin, von Nothnagel. See also Knapp's paper, cited below. 

79 Boston Med. and Surg. Jour., September 27, 1894, p. 308. Knapp gives a 
bibliography including many of the reported cases of recurrent oculomotor palsy 
up to that date. 

80 Recurrent Ptosis, in N. Y. Med. Journ., 1897, No. 7. 

81 See the case, reported by the present writer, of unilateral complete and total 



OPHTHALMOPLEGIC MIGEAIJSTE 719 

Some authors speak of recurrent oculo-motor palsy without pain, and 
cases have been reported, but they are probably not migrainous. 82 In 
fact there is not a little chance of confusing cases of organic paralysis 
of the third nerve with cases of migraine. This subject will be referred 
to more at length presently. 

Troemner 83 has reported a rare case, namely, ophthalmoplegia in- 
terna following severe attacks of migraine. There was no palsy of the 
extraocular muscles. The right pupil was widely dilated and immobile 
to light. 84 

Diagnosis. — Paralysis of the oculo-motor nerve is not an uncommon 
result of organic diseases of the brain, among the most common of which 
are brain-tumors and brain-syphilis. Both these lesions are productive 
of pain, and in both of them the palsy of the third nerve may be 
paroxysmal, although this is less likely to be so with tumors than with 
syphilis. The latter affection is indeed very apt to cause oculomotor 
palsy. It was a favorite saying with Picord that syphilis puts its sign 
manual on the third nerve. Thus an error in diagnosis might easily 
be made in cases which at first view may appear to be migrainous. 

In order to establish the differential diagnosis the history and course 
of the case must be considered. In cases of brain-tumor the symptoms 
are not usually limited to the third nerve ; there are other evidences of 
intracranial disease, such as choked disk, possibly convulsions, and other 
and more widespread paralyses. 85 Moreover the symptoms do not inter- 
mit so markedly as in migraine ; the course is steadily progressive. 86 

ophthalmoplegia, with anaesthesia of the fifth nerve and optic atrophy, in a Avoman 
with syphilis. (Univ. Med. Mag., October, 1899.) 

It is well also to be on guard against a mere hysterical anaesthesia in the terri- 
tory of the fifth nerve. The present writer has reported (Phila. Med. Journ., March 
30, 1901) a case of Bell's palsy in which the whole side of the face and head was the 
seat of an hysterical anaesthesia. It is to be noted in such cases that the anaesthesia 
is not confined strictly within the limits of the fifth nerve, for it is a peculiarity of 
hysterical anaesthesia everywhere not to be located within the exact distribution of 
any particular nerve. 

s2 Schmidt-Rimpler, Handbuches von Nothnagel, Bd. xxi. 

83 Cent. f. Nervenheilk., October, 1899. 

84 The following papers on the general subject of ophthalmoplegic migraine have 
not been obtainable: Marina, Ueber Multiple Augenmuskellahmung. Wien, 1896; 
Chiarini, La Emicrania Optalmoplegica, Rif. Med., 1895, xi. 109; Graef, Mauth 
Vortrag. xiii. 398. 

85 Richter (Arch. f. Psych., 1887, Bd. ix. p. 259) records a case of recurrent 
palsy of the third nerve caused by a fibrochondroma of the dura involving the nerve. 
There were epileptic attacks, and the case differed otherwise from the syndrome of 
ophthalmoplegic migraine. See also Karplus (Wien. klin. Woch., 1895, No. 50). 

86 For a case of brain-tumor causing paralysis of the third nerve see a report by 
the present writer (A Case of Tumor of the Mid-Brain and Left Optic Thalamus, 



720 THE EYE AND NERVOUS SYSTEM 

In the case of brain-syphilis the difficulty of making a diagnosis may be 
greater, and in fact some of the reported cases of recurrent oculo-motor 
palsy have probably been of syphilitic origin. The history of many 
preceding attacks, always the same, would be in favor of migraine, as 
would also the transitory nature of the attack in most cases. Optic 
neuritis may or may not be present in brain-syphilis ; it is never seen 
in ophthalmoplegic migraine. In case the history is obscure great care 
should be exercised in making this diagnosis. 87 

The nuclear ophthalmoplegia, described by Hutchinson, is an insidi- 
ous and progressive disease in which as a rule one nucleus, and there- 
fore one nerve branch, after another is involved, and in which there is 
not a tendency to periodical recurrence and recovery. Moreover the 
affection is usually bilateral, and not accompanied with pain, although 
in the case reported by the present writer pain was a symptom. 

A history of syphilis can be elicited in some of these cases of ophthal- 
moplegic migraine, 88 but that is not necessarily a proof that this affec- 
tion is syphilitic. In the great majority of cases there is no such history 
recorded. 

A basilar pachymeningitis has been known to cause a one-sided 
oculomotor palsy with symptoms resembling migraine. 89 The same has 
been observed in a case of tubercular meningitis by Weiss, 90 in whose 
case there was tuberculosis of both lungs, and a recurrent palsy, the last 
time permanent, of the left third nerve. Wadsworth 91 records a case 
associated with otorrhea, but the association was probably a mere coin- 



in the Med. News, January 30, 1892, p. 129). In this case there was a crossed 
paralysis, — i.e., the third nerve on the side of the. lesion and the arm and leg on the 
opposite side were involved. This crossed paralysis, due to a lesion in the mesen- 
cephalon, has been called by the French the syndrome of Weber. It is signifi- 
cant of an organic lesion, although it may be closely simulated by hysteria. 

87 The present writer has recorded three cases of ophthalmoplegia of various 
kinds, all of organic origin. The first was a case of total and complete unilateral 
ophthalmoplegia (interna and externa) caused by a syphilitic neoplasm at the base 
of the brain just behind the orbit. (Annals of Ophthalmology, January, 1898.) The 
second was a case of nuclear ophthalmoplegia in a woman, closely following the type 
described by Hutchinson. In this case the palsy was not unilateral, neither was it 
total ; that is to say, not all the orbital muscles were involved. ( Philada. Med. 
Journ., June 3, 1899.) The third case was one of unilateral ophthalmoplegia, total 
and complete, associated with anaesthesia in the fifth nerve and atrophy of the optic 
nerve — probably caused by syphilis. (University Med. Mag., October, 1899.) 

88 Mengazzini, cited by Mobius, records a syphilitic case. See also Leaffer, Berl. 
klin. Woch., 1900, No. 30, p. 657. 

80 Gubler (Dis. lnaug., Dresden, 1897). See also Ziehen (Neurol. Centblt., 1899, 
xviii. 4, p. 173). Also de Schweinitz's case (Philada. Polyclinic, vi. 39). 
U0 Wien. med Woch., 1885, 17. 
o: Trans. Am. Ophthal. Soc., 1887, iv. 460. 



OPHTHALMOPLEGIC MIGKAISTE 721 

cidence. The periodicity of some of these cases has naturally suggested 
malaria as a cause, 92 but the relationship is not clear; although the 
present writer has seen a case of transient paralysis of some of the 
ocular muscles in a well-marked case of malarial poisoning in his service 
at the Methodist Hospital. Kemak 93 has recorded a case of ocular 
palsy in a case of juvenile muscular atrophy, but it has no bearing on 
the subject of migraine. Thompson 94 had a case associated with con- 
vulsions and dementia in a child aged 5 years, and one of Joachim's 
, cases was in a backward child who also had epileptiform convulsions. 95 

In a few cases of recurrent oculo-motor palsy there has been noted 
a nasal catarrh. This was seen in Knapp's case ; also in a case recorded 
by Ahlstrom. 96 The latter observer thought the catarrh was the cause 
of the palsy, which he believed was " reflex." But the query arises 
whether the catarrh may not be an effect of the involvement of the fifth 
nerve supply to the mucous membrane of the nose. The symptom has 
not been recorded in many cases. Mobius 97 and # Bernhardt 98 suggested 
an " auto-infection" as the cause, or it may be " rheumatic." These 
ideas are merely speculative. 

In a case recorded by Karplus in which the attacks occurred as a 
rule every two or three months, no seizures occurred during pregnancy. 
This exemption is analogous to what is seen in epilepsy in not a few 
cases. 

Some observers have seen a relationship between tabes dorsalis and 
migraine; a relationship which is suggested especially by recurrent 
palsy of the third nerve in locomotor ataxia. Thus Oppenheim 99 in 
85 cases of tabes found 12 cases with attacks of migraine, only two of 
which, however, were of the ophthalmoplegic type. He claimed that 
migraine is a frequent symptom of beginning tabes. But it seems of 
doubtful propriety to call the recurring palsies of the third nerve in 
tabes instances of migraine. They are due to the pathological process 
underlying the tabes, and the history and clinical course are not the 
same as in migraine. It is desirable, moreover, to avoid mistakes, 
such as overlooking the onset of locomotor ataxia, and calling a tabetic 
third-nerve palsy a symptom merely of migraine. The same may be 

92 Klialschkin, Neurolog. Centblt., 1897, xvi., 5. 
93 Neurolog. Centblt., 1884. 

94 Char. Ann., 1885, p. 67. 

95 Jahrb. b. Kinderheilk., Bd. xxviii. p. 101. 
90 Ophthal. Rev., xv. 191. 

"Berl. klin. Woch., 1884, 38. 
98 Ibid., 1889, 33. 
"Ibid., 1884, 38. 
46 



722 THE EYE AND NERVOUS SYSTEM 

said of general paresis, of which disease also Oppenheim 10 ° sees an 
early symptom sometimes in ophthalmic migraine. As in the ophthal- 
mic type of migraine, so in the ophthalmoplegic type, hemiparesis and 
aphasia have been noted. 101 It is well to be on the lookout in such cases 
for hysteria. 

As already said (page 709) hysteria may complicate migraine, but 
the instances in which hysterical stigmata have been noted in ophthal- 
moplegic migraine are not numerous. Hasner 102 had a patient who 
every month had a paralysis of the oculo-motor nerve, lasting three days, 
and coinciding with the appearance and disappearance of the menses, 
but this fact, although it suggests, does not prove hysteria. Jacobi 103 
has reported a case of hysterical paralysis of the right third nerve, 
which was complete except in the superior oblique and the pupil; the 
latter, while it was dilated, would react slowly to light. There was no 
migraine, and cure was obtained by suggestion. In studying hysterical 
affections of the muscles of the eye it is important not to confuse paraly- 
sis with blepharospasm. The latter is much the more common. 104 Hys- 
terical affections of the eye and of the eye-muscles are very numerous 
and not a little complicated, and some of them may simulate the eye 
symptoms of migraine. This is true especially in cases in which there 
is an hysterical amaurosis combined with either a blephorospasm or a 
spastic strabismus simulating paralysis of some of the muscles supplied 
by the third nerve, or possibly the sixth nerve. 

Hysterical blindness or amaurosis is a rare affection; in this con- 
dition there is loss of vision. But a much more common affection is 
a contraction of the visual fields, which might simulate a scotoma to a 
careless observer. In hysteria there is also a reversal of the normal 
color fields, the field for red being larger than the field for blue. These 
hysterical affections of the visual fields are always associated with other 
hysterical stigmata, and they are essentially unlike the visual aura of 
ophthalmic migraine. In hysteria there is not the scintillating scotoma, 
and the disorder in the visual field is rather a contraction than a true 
scotoma. In some very rare cases (one of which has been put on record 
by the present writer 105 ) a true hemianopsia has, however, been ob- 

100 Char. Ann., 1890, Bd. xiv. 

101 Hinde and Moyer, N. Y. Med. Rec, 1887, 418. 

102 Prag. med. Woch., 1883, No. 10. 

103 Am. Journ. Obstet., 1876, p. 223. 

104 Gilles de la Tourette, Hysterie, chap. ix. The subject is discussed at great 
length by Tourette, who seems to be sceptical about a hysterical paralysis of the 
interior muscles of the eye. 

103 Article on Hysteria in Dercum's Text-Book of Nervous Diseases, by American 



PSYCHICAL MIGKAIKE 723 

served in hysteria. But the visual aura in migraine, as we have seen, 
is very transient, differing in this respect from the visual disorders of 
hysteria. 

As Tourette has pointed out in great detail, following especially 
Borel, 106 the muscles of the eye in hysteria are not so much the seat of 
paralysis as of spasm. In fact it may he a question whether there is 
a true hysterical paralysis of the third nerve, and whether the reported 
cases have not really heen instances of blepharospasm and of sj)astic 
strabismus. In that curious affection known as monocular diplopia, in 
which the hysterical patient sees double with one eye, the cause is prob- 
ably a spasm of the muscle of accommodation, causing irregular con- 
traction of the crystalline lens. 107 So in the pseudo-paralysis of the 
third nerve, studied especially by French observers, the affection is in re- 
ality a contracture of the orbicularis muscle causing an apparent droop- 
ing of the upper lid. Blepharospasm, or contracture of the orbicularis, 
is indeed the commonest hysterical affection of the eye-muscles. Iso- 
lated paralysis of any of the orbital muscles, and especially of the intra- 
ocular muscles, as of the iris or ciliary muscle, of hysterical origin, is 
probably never seen, although apparent cases have been reported; but 
the strabismus is probably always due not to a paralysis but to a spasm 
of one or other of the ocular muscles. This seems to be the conclusion 
of Tourette, who has traversed this subject with great care, and to Avhose 
work the reader is referred for details. 

From what has been written it is seen that the distinction between 
the ophthalmoplegia of migraine and the blepharospasm and spastic 
strabismus of hysteria is not a subject of great difficulty in the hands 
of a careful observer. 

PSYCHICAL MIGRAINE. 

Mental disorders of various kinds have been reported by many 
observers of migraine. The subject was discussed at length by Live- 
ing, 108 who gave many instances which he had culled from the literature. 

Authors, p. 112. In that article the hysterical affections of the eye are discussed in 
more detail than there is room for in the present paper. See Wecker et Landolt 
(Traite Comp. d'Ophthalmologie, article on Amblyopic Hysterique) for references 
to a number of cases of hysterical hemianopsia. 

106 Affections Hysteriques des Muscles Oculaires, Ann. d'Ocnl., Nbvembre, De- 

cembre, 1887. 

107 Tourette, op. cit. Also Parinaud, quoted by Tourette. 

10s Op. cit., p. 107. Liveing was a most diffuse writer and a great compiler, and 
some of his quotations are perhaps wide of the mark: but his passages on . the 
psychical disorders of migraine form an interesting contribution to this subject, 
and more complete than any other work of which the present writer lias knowledge. 



724 THE EYE AND NERVOUS SYSTEM 

He divided these disorders into two groups, the intellectual and the 
emotional. 

Lebert in describing his own symptoms said that his seizures began 
with some incoherence of ideas and difficulty in finding words. Others 
have described confusion of intellect and impairment of memory. Even 
a partial loss of consciousness has been experienced. Some of these 
affections of mind are accompanied with a sensation of numbness in the 
extremities, face, and tongue, and with an affection of the speech, which 
will be referred to again. Some permanent impairment of mind has 
been observed in migrainous patients who have suffered with severe 
and oft-repeated attacks. Elaborate descriptions have been indulged in 
by some of these patients, as one old man, referred to by Liveing, who 
said that as the visual phenomena passed off, his memory usually failed 
so much that for a time he was mentally incapacitated, and whatever 
he read or did during that period left no impression ; he experienced a 
singular disorder of ideation ; circumstances and events of long before 
were brought back to him as if actually present ; his consciousness 
appeared to be doubled, and the past and present were confused. 

Emotional disorders are not uncommon during the first stage of 
migraine. The state of mind in some patients is one of fright or great 
dread. They know what is in store for them and fear it; but the 
apprehension or demoralization seems in some cases out of proportion 
to the cause. This has been acknowledged by migrainous patients them- 
selves. 109 The visual aura, with its ugly scintillating scotoma, is par- 
ticularly disturbing, and quite unnerves some individuals ; so much so 
indeed that they avoid speaking or even thinking of it during the inter- 
vals, as in a case referred to by Airy. This vague and unaccountable 
fear supplies another analogy with epilepsy, in which, as is well known, 
a sense of terror is often experienced during the brief aura. 

As we have said before, this mental state of the migrainous patient 
is well adapted to excite hysterical symptoms, and it is rather remarka- 
ble that more has not been written on this topic. Possibly some of the 
anaesthesias, tinglings, and speech defects are truly hysterical, but there 
is a dearth of cases in which the subject of hysterical stigmata has been 
put to the test. The severe suffering of the patient, following so soon 
on the aura, may mask such symptoms, and does not invite to a close 
examination for them. 

Mental depression, not only during the aura, but even between the 



109 An early writer who recognized this inordinate dread was Dwight, who gave 
a good description. (Medical Repository, vol. ii. p. 16, New York, 18t)0.) 



PSYCHICAL MIGKAIKE 725 

paroxysms, is present in some patients. It may be explained by the 
great suffering and the frequent incapacity which results from the 
attack. 

In endeavoring to explain the mental symptoms in migraine two 
points of view present themselves. First, the psychical disorder may 
be due to the dread of the approaching suffering and to the suffering 
itself. Great pain, frequently repeated, is demoralizing to the human 
soul. The ancients said that physical pain was the greatest evil that 
man could endure. Second, the mental symptoms may be looked upon 
as an integral part of the nerve storm. This seems to be true from 
the fact that strong-minded men have endeavored in vain to control the 
emotional depression, the fear and anxiety, which sometimes precede 
the onset of the pain. In other words, there is a true psychosis attend- 
ant upon the attack of migraine. 110 

Disorders of speech have also been noted by not a few observers as 
occurring in migraine. Liveing m has collected much curious informa- 
tion on this subject. Oppenheim 112 says that he has observed aphasia, 
combined with agraphia. Word-deafness has been observed in only a 
few cases. The disturbance lasts from a few minutes to half an hour, 
and is succeeded by the headache, which is always upon the left half 
of the head. This fact, of course, suggests that the speech defects 
are due to a temporary affection of the speech centres in the left cere- 
brum. What this affection is must be as yet a mere matter of conjec- 
ture; but it seems necessary to suppose that it is some material affec- 
tion, something acting to disturb possibly the blood supply or the nutri- 
tion of the brain cells of the speech centres in the cortex. Liveing, 
although he wrote before much was definitely known about the speech 
centres, saw the possible significance of these speech disorders, and col- 
lected a number of cases which tended to show that the migraine in these 
instances was on the left side of the brain. He noted that migraine is 
often a one-sided affection, and the thought occurred to him to investi- 
gate whether the left side was most often affected in these cases. Thus 
he found that in Lebert's own case the speech disorder was accompanied 
with a numbness in the tongue and fingers of the right hand. In sev- 
eral other cases the phenomena were similar; there was speech disorder, 
with numbness on the right side, as in the hand and arm, neck and face. 

110 Oppenheim, Dis. Nerv. Syst., p. 751. The subject has been recognized also 
by Griesinger and Krafft-Ebing. The psychosis according to these observers is one 
of excitement, confusion, or stupor. 

111 Op. cit., p. 93. Localizing data are still wanting in migraine. See ante, 
p. 10. 

U2 Op. cit., p. 751. 



726 THE EYE AND NEKVOUS SYSTEM 

In Abercrombie's case the numbness (aura) extended upwards from the 
fingers of the right hand, and was followed by impairment of speech. 
Some of these cases were very striking, and seem to be more than mere 
coincidences. Of 15 cases thus analyzed by Liveing, in 7 the numbness 
was on the right side ; in 4 it was bilateral, while in the remaining cases 
the report was defective. But in no single instance was the left side 
of the body alone affected. The inference seems clear that the 
speech defects in migraine are often caused by a left-sided lesion in 
the brain. 

In some cases reported as migraine with aphasia it is possible that 
Bright's disease may have been a factor, for this affection may cause 
temporary aphasia with headache. The writer has seen a well-marked 
example, simulating tumor of the brain, in his service at the Philadel- 
phia Hospital. 

But if we are to follow out the analogy of migraine with epilepsy, 
we should seek for a true psychical migraine, not in the- mere presence 
of psychical symptoms, but in the substitution of such symptoms for 
the classical migrainous attack. Are there such cases of pure psychical 
migraine ? The answer seems from a few recorded cases to be in the 
affirmative. Sir George Airy 113 observed in his own person the usual 
attack complicated with transient impairment of speech and memory 
without either headache or numbness. Parry 114 advanced the theory 
of a compensation between the different phenomena of the seizure ; if 
the disorder of sight proved abortive, the higher cerebral faculties were 
more likely to be involved. Tissot, quoted by Liveing, related a case in 
which attacks of habitual migraine were at length completely replaced 
by fits of disordered ideation — a true substitutional attack, or pure psy- 
chical migraine. We are indebted to Liveing for more information 
on this obscure subject than is supplied by most later writers. 

Among ojher mental changes is the state known as double-conscious- 
ness. 115 Vertigo is also sometimes experienced. These symptoms indi- 
cate how profoundly the mental or cerebral functions may be affected 
in migraine. 116 

Treatment. — The treatment of migraine is entirely symptomatic 
and expectant. There is no specific for the disease. In our ignorance 
of the pathology of the affection, our treatment can be nothing more 

113 Philosophical Magazine, July, 1865. 

114 Pathology and Therapeutics, vol. i. p. 357, quoted by Liveing. 

115 Gowers, Dis. Nerv. Syst., vol. ii. p. 842. 

116 See also Bounal for a case with epileptoid symptoms, Rev. Mens, de Med. et 
Chir., 1878, ii. 279; Gray, The Correlation and Interconvertibility of Migraine and 
Epilepsy, Pathologist, 1881, i. 4. 



PSYCHICAL MIGRAIKE 727 

than empirical; and this fact is proved, as in all similar cases, by the 
large number of remedies that are recommended and even lauded in 
excess. From quack nostrums to spectacles the list is a dreary one, and 
will not be repeated here. 

We may, however, consider briefly a few of the more approved rem- 
edies. From the writer's personal experience in his own case it has 
seemed that an abortive treatment is always worth a trial. This treat- 
ment consists in prompt action on the gastro-intestinal tract. A saline 
laxative, such as Rochelle or Epsom salts, taken at the first appearance 
of the visual aura, will sometimes cut short an attack. If free purgation 
is obtained in an hour or two, the attack may be aborted or its severity 
diminished. The same is true of an emetic, especially if the attack 
comes on soon after the patient has taken a hearty meal. Under such 
circumstances I have known a full dose of ipecac promptly to abort the 
attack. As soon as the stomach is emptied the hemicrania diminishes 
and soon ends. Although the remedy is not pleasant, it is pleasanter 
than a long period of sick headache with the inevitable emesis that 
comes anyhow at the termination of the attack. It is but proper to say, 
however, that in some of the more severe cases these remedies addressed 
to the stomach and bowels will not bring entire relief. 

The symptomatic treatment is usually addressed to the relief of 
pain ; and here the extensive list of anodyne drugs comes into play. A 
popular migraine powder at present is composed of citrate of caffeine, 
monobromide of camphor, and acetanilid. The various coal-tar products 
are often appealed to, but seldom do more than assuage the pain. They 
are not curative, and they are therefore often disappointing. Among 
them are antipyrine, antikamnia, and phenacetine. 

Cannabis indica was long regarded with favor in migraine, and 
it is extolled by Sinkler 117 in his excellent discussion of treatment. 
Of recent years, however, we hear little of it. Remedies which are 
supposed to control the vasomotors, such as ergot, have also been com- 
mended, but on purely theoretical grounds. The victim of migraine 
who puts his trust in them will suffer for his faith. Nitrite of amyl is 
said to give relief in the early stage. The bromides have been used 
extensively, not without temporary relief. 118 So, too, of chloral, and 
croton chloral. Opiates, and especially the hypodermic use of morphia, 

117 Article, Migraine, in Pepper's Syst. of Med., vol. v. p. 413. Also, by the same 
author, in Hare's Syst. of Pract. Therapeutics, vol. iii. p. 375. 

ns Tourette et Blocq (Prog. Med., xv. 47G), in a case of ophthalmic migraine 
with transitory aphasia, obtained good results with bromides, which they used in 
doses as high as 5 grammes a day. 



728 THE EYE AND NERVOUS SYSTEM 

will deaden the pain, but the practice of giving morphia in this way to 
patients who have frequent attacks, is most objectionable. The risk of 
forming the habit is not small. Local applications of all sorts have been 
used ; heat, or its converse, cold, is probably among the best. 

The expectant treatment, or that which is applied to warding off 
the attacks, consists merely in a tonic, hygienic, and restorative regime. 
The usual, and sometimes useful, drugs for this purpose are quinine, 
iron, mix vomica, arsenic, and phosphorus ; but it is the irony of 
therapeutics to recommend these drugs as in any sense curative. 

Some patients, from long experience, find some particular drug or 
method best suited to their own cases, but the fact that such approved 
remedies are not universally effective proves how much the personal 
equation rules in migraine as in other neuroses. It is still a disease 
that favors experimental therapeutics, arid the stage of scientific achieve- 
ment has yet to be reached. 

TETANUS. 

The eye-symptoms in tetanus are few in nurnber. In ,the ordinary 
form of the disease these symptoms are practically nil, but m that rare 
and interesting kind of lockjaw known as cephalic tetanus (tfhe Tcopf- 
tetanus of the Germans) there is occasionally seen a paralysis' of one 
or more ocular muscles. 

In cephalic tetanus the disease usually takes its origin in a wo^und 
situated in the distribution of the fifth nerve. The tetanic symptoims 
may be limited to the head, throat and neck, although in some easels 
there is also opisthotonos, and even general tetanus. The peculiarity of", 
this form of tetanus is that there is an associated paralysis of the seventh 
nerve. The muscles of deglutition are involved, a fact which led Rose 
to call the disease tetanus hydro phobicus. But this is an unfortunate 
name, for the affection has no relation whatever to hydrophobia. 

Cephalic tetanus is a rare affection. But few cases have been 
recorded in America. The most complete account of the disease ever 
given in this country was published by Willard 119 in 1895. That 
report was based upon the history of a case in a boy, whose attack was 
caused by a wound, from a dirty stick, in the neighborhood of the orbit, 
and who made a good recovery. 

Willard collected the references to 74 cases of cephalic tetanus, his 
own being the seventy-fifth case. There were 57 males and 15 females 
noted. In 45 early cases, in which the tetanus developed within the 

119 Tetanus Cephalic, etc., Trans. Col. Phys. of Philada., 1895, 3d Series, vol. 
xvii. p. 27. 



TETANUS 



729 



first week, 39 died and I recovered. In 32 later cases, in which the symp- 
toms developed after the first week, 8 died and 24 recovered. These 
figures illustrate the fact that the mortality from cephalic tetanus is 
rather less than from the ordinary form, and that the prognosis is much 
more favorable in the late cases. 

The ocular muscles have been involved in a very few of these cases 
of cephalic tetanus. In the first place, of course, the orbicularis muscle, 
which is supplied by the seventh nerve and is not properly an ocular, or 
at least an orbital, muscle, is involved along with the facial muscles 
supplied by the facial nerve. The eye is open, as in all cases of periph- 




Fig. 3.— Cephalic tetanus, with paralysis of both seventh nerves. This picture shows the patient 
in the attempt to close her eyes. Trismus. Ironed-out expression. Wound on nose. The eye muscles 
were not involved, although the right eyeball rolled unduly inward in the attempt to close the eyes. 



eral palsy of the seventh nerve, a fact which shows that the paralysis 
is peripheral. There has been some discussion as to the exact pathology 
of this peripheral palsy; in some cases examined post-mortem the evi- 
dences of neuritis have not been found ; and during life the reactions of 
degeneration have not been marked. In Willard's case the present 
writer studied the electrotonus, and found no reactions of degenera- 
tion, although the examination was incomplete owing to the patient's 
excitement and resistance. 

In a very few cases of cephalic tetanus some of the orbital muscles 



Y30 THE EYE AND NERVOUS SYSTEM 






have been involved along with the facial muscles. In one case ptosis 
was observed. 120 In another case 121 paralysis of the external and 
internal ocular muscles of one side, with facial paralysis of the oppo- 
site side, was seen. Willard makes no reference to this complication, 
so it is probable that it has existed in rare instances only. V. 
Schrotter 122 has put on record a case of kopf tetanus in which there 
was paralysis of the facial, the ocular, and the hypoglossal nerves. 
Neumann 123 says that the facial nerve is paralyzed first ; then the 
oculomotor, abducens, trochlear and hypoglossal are affected in the 
order named. 

The pathology of this affection, as already said, is obscure. Tetanus 
is caused by a bacillus, and this is no doubt so also in the cephalic form. 
But ordinary tetanus does not cause paralysis ; quite the reverse. We 
are driven to the conclusion, entirely speculative, that for some reason 
the poison of tetanus, especially when introduced in the area of the fifth 
nerve, has the power of paralyzing some few of the cranial nerves, 
especially those supplying the face and the orbit, but not exclusively 
these, for the hypoglossus also may be involved. It is possible, as 
has been suggested to the minds of several observers, and has been 
voiced by Neumann, that the poison acts upon the nuclei of the 
nerves involved. This seems to be shown by the absence of the post- 
mortem evidences of neuritis, as well as the absence of the reactions of 
degeneration. 

TETANY. 

In this disease, which is not common in America, eye symptoms 
seem to be rare. Of recent European writers Eilia, 124 who reports 44 
cases, makes no mention of such symptoms. The same is true of 
Pons. 125 Another observer, Loos, 126 has observed nystagmus in a child. 
The fundus oculi is usually normal, although Miiller 127 described a 
case with neuro-retinitis and later beginning atrophy ; but the case was 
complicated with scarlet fever. V. Jaksch 128 had a case with double 
choked disk and diplopia, but other symptoms pointed to an organic 



120 Serein, L'Union Med., 1886, No. 173. 

121 Roberts, Lancet, July 11, 1891. 

122 Fall von Kopftetanus, in Wien. klin. Woch., 1902, No. 2, p. 56. 

123 Ein Fall von Kopftetanus, in Zeit. f. Heilkunde, 1902, p. 344. 

124 La Tetanie chez les Enfants, in Rev. d'Hyg., 1903, ii. 505-537. 

125 Sur Quelques Points de la Tetanie Infantile, Toulouse, 1903. 

126 Deut. Arch. f. klin. Med., Bd. 1. S. 169. 
127 Deut. Milit. Arzt. Zeits., 1884, xiii. S. 439. 
128 Zeit. f. klin. Med., 1890, xvii. 144. 






EPILEPSY 731 

brain lesion. Marschner 129 describes a case of neuroretinitis in tetany, 
but the patient had also pleuro-pneumonia. Cassel 13 ° describes a case 
of the disease with optic atrophy, but gives no other details regarding it. 

Kursmal 131 observed a case in which the pupils were unequal. 
There is sometimes a cramp in the eye muscles. This is usually a 
blepharospasm. There may be also a sudden convergent strabismus 
with diplopia. The tetanic spasm of the eye muscles may be accompa- 
nied with pain, reddening of the conjunctiva?, and lachrymation. 132 
Cases have been described in which the eye symptoms appeared alone, 
or alternating with the other symptoms. The pupil is seldom dilated, 
and even more rarely contracted. 133 

These few references to the disease, by recent European writers who 
see much of it (and much more than is seen in America) seem to indi- 
cate that the ocular symptoms, although not lacking, are rather rare. 
American and English writers (Osier, Dana, Gowers) make but little 
if any reference to these symptoms, and only to the contractions in the 
eye muscles. 

EPILEPSY. 

The ocular symptoms of epilepsy are important from a diagnostic 
stand-point. They have also in some cases not a little medicolegal sig- 
nificance. In considering them it is necessary to remember that they 
are usually of a transient character, and that to detect them requires 
alertness and some skill in observation. (See Chapter XVIII, p. 782.) 

These symptoms may be divided conveniently into groups. Thus 
they are visual, muscular, and vascular. It is desirable to state at once 
that it is not the object of this paper to describe epilepsy itself, but 
merely its ocular symptoms. Only so much of the disease will be 
referred to as is necessary for an understanding of these symptoms. 
Epilepsy, like migraine, is an explosive neurosis, preceded in the vast 
majority of cases by an aura. Its ocular manifestations are sometimes 
a part or even the whole of the aura ; while in other cases the involve- 
ment of the eye is in the nature of a sequel. In the majority of cases, 
however, it is either as an aura, or as a manifestation of the early stage 
of the fit, that the eye symptoms are prominent. 

The purely visual symptoms of epilepsy are of course entirely sub- 
jective, and we must therefore rely entirely on the patient's own state- 

129 Deut. Arch. f. klin. Med., 189G, Bd. lvi. 501. 

130 Berl. klin. Woch., 1896, 69. 

m Deut. Arch. f. klin. Med., 1869, vi. 481. 

182 Hochwart, Tetanie, in Nothnagel's Spec. Path. u. Ther., Bd. xi. p. 134. 

133 Verein f. Psych, in Wein, 1895. 



732 THE EYE AND NERVOUS SYSTEM 

ments for a knowledge of them. 134 They may appear as an aura or as 
a sequel. It is in the nature of the aura? of epilepsy, unlike those of 
migraine, to be very fleeting, even instantaneous ; and when these aura? 
are visual they consist usually of mere momentary affections of sight. 
Some of them are not unlike the visual aurse of migraine ; a fact which 
has led some observers to see a more intimate relationship between these 
two diseases than perhaps the facts warrant. 

Mere obscuration of vision, something like the amblyopia of mi- 
graine, is an epileptic aura in some cases. 135 A true scintillating 
scotoma, somewhat like that seen in migraine, is claimed by Bens- 
wanger 136 to appear sometimes in epilepsy, but this is rare; and it is 
difficult to understand how an epileptic patient would have time ordi- 
narily to note the characteristics of such a sign. Fere 137 also refers to 
obscuration of vision as a symptom of epilepsy. Some patients say 
that everything becomes black before them just before the seizure ; by 
this they probably indicate a visual aura, or amblyopia. Herpin, 138 
who has reviewed the history of epileptic aurse from the time of Hip- 
pocrates, gives no instance of a true visual aura, except in a case re- 
ported by Maissonneuve, 139 in which the attack began with a dazzling 
sensation (eblouissement) in the right eye, which continued for some 
minutes after the return of consciousness. The original idea of an 
epileptic aura was that it was caused by a " vapor" extending up the 
vessels of a limb ; and indeed the word " aura" is derived from the 
Greek word meaning " a vapor." This idea was so firmly fixed that it 
dominated the medical world, as Herpin shows, even until very modern 
times, and to it may be ascribed the fact that the older observers were 
not on the lookout for any kind of aura except the sensory warning 
beginning at an extremity. Hence aura? of the special senses were not 
recognized by them, because, forsooth, they were not mentioned by 
Galen and the ancients. But in recent times auras of the special senses 
have been fully recognized, and, as Gowers 14 ° remarks, a visual warn- 
ing is twice as common as all the other special sense aurse together. 
This visual aura may be a sudden loss of sight, or it may be a flash of 
colored light or other chromatic appearance. 

134 Spratling ( Epilepsy and its Treatment, p. 226 ) gives a long list of the 
visual aurse which he has noted in his patients. 
133 Voisin, Les Epilepsies, 1896. 

136 Die Epilepsie, in Nothnagel's Spec. Path. u. Therap., Bd. xii. 

137 Les Epilepsies, Paris, 1890. 

138 L'Epilepsie, Paris, 1852, p. 389. 
130 Cited by Herpin, op. cit., p. 411. 
140 Dis. Nerv. Syst., vol. ii. p. 739. 






EPILEPSY 733 

Flashes of light or balls of colored or uncolored light are among 
the commonest visual aurse of epilepsy. They are variously described 
by patients, and doubtless they vary in different cases. There may be 
merely a glare, a flash, or a dazzling sensation of light, and this may 
be colorless or it may be distinctly chromatic. Red seems to be the 
commonest color. In some cases these brilliant aurse take distinct 
shapes, especially that of a ball, and this may be seen approaching or 
receding, growing larger or smaller. This tendency to increase or 
diminish in size, to advance or recede, is sometimes seen in other objects, 
as will be referred to presently. Gowers, 141 who seems to have had the 
fortune to observe more complicated cases of visual aura? than most 
other observers, describes instances in which these visual warnings were 
associated with other aura?, such as of taste, smell, and common sensa- 
tion. He also calls attention to the fact that the various colors of the 
spectrum are not seen in the same frequency. Thus they appear in 
the following order, red, blue, green, yellow, and purple, which is not 
quite the physiological order in which the colors are represented in 
the normal visual fields, for blue occupies the larger field in the normal 
eye. There is some suggestion here of what occurs in hysteria — that 
is, a reversal of the color-fields — red occupying the most prominent 
place instead of blue. Voisin also says that red is the color most fre- 
quently seen in the epileptic aura. 

A curious form of epileptic aura is that in which objects appear to 
grow larger or smaller, as the case may be. In the one case they may 
appear to approach, in the other to recede, or even to grow thicker or 
thinner. These phenomena have been called respectively macropsia 
and micropsia. 14 ' 2 Patients have described the objects in a room as 
receding to a great distance. In other cases they appear to approach 
and enlarge until consciousness is lost in the fit. 

Sometimes, as Eere points out, 143 there is a photophobia or dyses- 
thesia of the retina: the patient cannot bear the light, and there is a 
sense of pain in the eye with a forcible reaction of some of the ocular 
muscles, especially a blepharospasm. Eere and others also refer to a 
dyschromatopsia, or inability to distinguish colors ; but it is difficult 
to understand how such fine tests can be made in the brief period of 
an epileptic aura. Dyschromatopsia and changes in the visual field, 
such as contraction and reversal of the color-fields, may persist as post- 



141 Epilepsy and Other Chronic Convulsive Diseases, 2d Ed., p. 74 et infra. 
142 Veraguth, Ueber Mikropsia und Makropsia, Deut. Zeit. f. Nervenheilk.. Bd. 
xxiv., H. 5, 6. 

143 Op. cit., p. 78. 



734 THE EYE AND NERVOUS SYSTEM 

epileptic phenomena, and these must carefully be distinguished from 
hysteria. In fact, various hysterical stigmata may complicate cases of 
genuine epilepsy, as the present writer has noted in the Philadelphia 
Hospital more than once.' 

The visual aura is sometimes located by the patient on one side, as 
in a case reported by Eere, 144 in which the patient saw a red mist before 
the right eye. In such a case it is a question whether the phenomenon 
is not hemiopic, — that is, seen in the corresponding halves of both 
retina?. For instance, when the object is seen apparently only by the 
right eye, it is really perceived by the left half of the retina in each 
eye. 145 

Spratling 146 speaks of a retinal epilepsy, in' which there is sudden 
blindness of brief duration without convulsion ; in other words, a sort 
of substitutional attack. The term " epileptic amaurosis," used by 
Hughlings-Jackson, has also been used by some writers in this sense. 

Of all visual aura? the most complex are the true hallucinations, in 
which, as it were, a panorama is unfolded before the patient's gaze. 
A few such cases have been reported. Gowers 147 describes minutely 
a complicated aura in which the patient saw an old woman in a brown 
dress, and Gregory's patient saw an old woman, too, but she was dressed 
in red. 148 These visions are sometimes of disagreeable or ugly objects 
or animals ; but occasionally the scene recalls events of long ago, as is 
sometimes seen in migraine also. There may be hemianopic hallu- 
cinations. 149 

The muscular affections of the eye in epilepsy may be observed in 
either the extra- or intraocular muscles or in both. It is not unusual 
in the epileptic fit for the eyes to be turned forcibly in one direction, 
as, for instance, upward or to one side. When the eyes are strongly 
rotated to one side it is usually in cases in which there is forced rotation 
of the head also to one side, and the eyes are usually rolled towards the 
same side as the head. Onuf 150 claims that there are exceptions to 
this rule, and that the eyes may be rolled to the opposite side from 
that towards which the head is rotated, but such exceptions must be 
rare. This lateral deviation of the head and eyes in epilepsy may pos- 

144 Op. cit., p. 464. 

145 Harris ( Hemianopsia, in Brain, 20, 307 ) refers to temporary hemianopia 
in epilepsy. 

146 Epilepsy and its Treatment. 

147 Epilepsy, etc., 2d Ed., p .78. 

148 Cited by Benswanger, op. cit. 

149 Harris, op. cit. See also ante, p. 11. 

150 New York State Med. Journ., 1904, iv. 71-74. 



EPILEPSY 735 

sibly indicate that one cerebral hemisphere is more involved in the 
epileptic discharge than the other, and it is the opposite of what is seen 
in gross cerebral hemorrhage in which there is also lateral deviation 
of the head and eyes, but towards the lesion, — i.e., away from the para- 
lyzed side. In epilepsy the deviation is usually towards the more con- 
vulsed side. 151 This is shown in cases of post-hemiplegic seizures in 
which the paralyzed side is the seat of the most active convulsions. 
Lateral deviation of the eyes, however, is not a localizing symptom of 
any great value in cerebral pathology. It occurs in too many conditions 
to be of special significance. 

Diplopia has been reported by some writers as occurring in the early 
stage of epilepsy. Fere and Gowers both speak of it. Strabismus and 
nystagmus have also been noted, both as accompaniments of the attacks 
and as sequellae of them. Diplopia is doubtless caused by a temporary 
strabismus which results from either a spasm or paresis of one or other 
ocular muscle. When it occurs as a part of the epileptic aura it is 
probably due to a spasm of some of the ocular muscles causing the two 
eyeballs to roll in different axes. Eere 152 relates a case in which an 
epileptic boy became suddenly cross-eyed in the interval between fits; 
this convergent strabismus, in the left eye, was increased after each 
seizure, at which time the patient saw double. The same author has 
noted permanent lateral nystagmus, also permanent blepharospasm fol- 
lowing epileptic seizures. Such permanent affections of the ocular mus- 
cles point to organic lesions, whether in the nature of an accident of 
the fit or as a cause of the fit, such as a tumor or a basilar meningitis. 
Speaking of nystagmus, Eere says it occurs towards the affected hemi- 
sphere in hemiplegic epilepsy in the initial stage. 

Much has been written about the state of the pupils during the epi- 
leptic fit. 153 It is commonly said that the pupil is dilated and unre- 
sponsive to light. Onuf says that there is extreme contraction of the 
pupil just as the attack begins, and that it is also contracted in the post- 
convulsive stage. Gowers confirms this statement with respect to the 
initial contraction, but says that dilatation quickly supervenes and is 
present during the tonic stage. He found, however, that in mild attacks 
the pupil is not always unresponsive to light. Reynolds 154 points out 
that at the end of the fit the pupils often exhibit marked oscillations. 
These oscillations consist of alternate contraction and dilatation, but are 

131 Fere, op. cit., p. 387. 

152 Op. cit., 129, also p. 177 et infra. 



Fere, op. cit., pp. 89, 178, 391, 417, 473. 
Epilepsy, 1861, p. 112. 



736 THE EYE AND NERVOUS SYSTEM 

not present in every case. The conjunctival reflex is also abolished dur- 
ing the profound unconsciousness of epilepsy : the eye may be touched 
or irritated with the finger without causing the usual reflex contraction 
of the orbicularis. In very mild cases of petit mal this may not always 
be so, doubtless because consciousness is not lost, or only momentarily 
so. 155 

The condition of the pupil has been appealed to in some cases as a 
medicolegal test. Evidently, in simulated epilepsy this state of dila- 
tation could not be feigned by an act of the will; but, as Voisin has 
pointed out, dilatation can be secured by the use of belladonna, and 
deception might thus be practised. Still, the action of belladonna or 
its alkaloid atropia is very persistent on the pupil, lasting for days, 
and this should be enough to expose the deception. 156 The dilatation 
in epilepsy, with abolition of the light-reflex, does not endure after the 
fit. But in a suspected case the dilatation with immobility of the pupil 
during the fit, speaks for epilepsy. 157 Fere speaks of asymmetry of the 
pupil in the epileptic fit; also of inequality of the pupils. 158 It is 
important to bear in mind that we are here discussing essential or idio- 
pathic epilepsy, — i.e., epilepsy without gross organic lesion. In cases 
of symptomatic epilepsy, as, for instance, in cases of tumors, hemor- 
rhage, or meningitis, the state of the pupils might be affected by such 
lesion more or less permanently and independently of the seizures. 

Vascular lesions in the eye are not common in epilepsy. Ecchy- 
moses or extravasations of blood beneath the conjunctiva are occasion- 
ally seen as results of the violence of the fit. 159 I have seen such a case 
in which a large part of the exposed surface of the eyeball was covered 
with extravasated blood. It is not a symptom of special importance, 
and it usually disappears in a few days. Congestion of the conjunctiva 
may be present during the seizure, but it soon subsides. These ocular 

135 Spratling (Epilepsy and its Treatment, 1904, p. 268) does not recall a 
single instance in which the pupils were not more or less dilated — in some cases 
extremely so. He does not believe that this is due to asphyxia, as some have held, 
because he has seen it in light attacks as a very early phenomenon. He thinks 
that inequality of the pupils points to an organic lesion, and thus it may persist 
during the intervals. 

150 See the Trial of Allen C. Laros, which took place at Easton, Pa., in August, 
1876 (reported by Edgar), for an example of alleged feigned epilepsy in which the 
state of the pupils was discussed. 

137 Max Bire, Ueber Epilepsie, Deut. Zeit. f. Nervenheilk., Bd. xxiii., H. 1, 2, 
p. 36. See also Hoche, Die differential Diagnose Zuischen Epilepsie und Hys- 
terie, Berl. Augen. Huschweld, 1902. 

158 Op. cit., 391. 

159 Voisin, op. cit. 



EPILEPSY 737 

hemorrhages are sometimes associated with hemorrhages in other parts 
of the body. 160 Retinal hemorrhage is rare. Gowers thinks it is pre- 
vented by the intra-ocular tension. The appearance of the fundus is 
not significant in ordinary essential epilepsy. Between attacks the 
fundus is normal, and during attacks the only marked change is in- 
creased fulness of the retinal veins ; this is in unison with the general 
congestion of the vessels of the head. When traces of old optic neuritis 
are found in so-called idiopathic epilepsy, they may be due to an old 
syphilitic lesion which may have acted as the original cause of the epi- 
lepsy, as Gowers has pointed out. Epilepsy itself does not cause per- 
manent changes in the fundus of the eye, as is the universal verdict. 
It is a rather curious fact that, as Eere points out, an ophthalmoscopic 
examination in an epileptic will sometimes excite a seizure. 

In convulsive attacks due to uraemia and to chronic lead-poisoning 
(encephalopathy) there may be well-known changes in the fundus due 
to those conditions, but such changes are in nowise either the causes 
or results of the fits. 

There are but few ocular sequellce of epilepsy. Mention has already 
been made of ocular palsies which may persist after the fit, but they 
are rare. In genuine psychical epilepsy, in which the convulsion is 
replaced by a psychosis, hallucinations of sight may occur ; also in the 
post-paroxysmal stage. 161 These hallucinations are usually of alarming 
objects, as ugly faces, devils, etc. 162 In testing the visual fields after 
an epileptic attack, it is important to remember, as already said, that 
such symptoms as dyscromatopsia, contraction of the visual fields, re- 
versal of the color fields, etc., may be of hysterical character, for it is 
not rare for epilepsy to be complicated with hysteria. The error must 
not be made, however, of supposing that because a patient has a few 
hysterical stigmata after a fit, therefore his attack is not genuine epi- 
lepsy. The two diseases may co-exist in one and the same patient. So, 
too, of migraine. The fact that a patient has visual aurse resembling 
those of migraine, or even the still more striking fact that a patient 
has both migrainous and epileptic seizures, is no reason for claiming 
that those two diseases are so intimately related as is done by some 
authors, conspicuous among whom are both Gowers and Fere. 163 



160 Spiller, Uncinate Group of Fits, Am. Med., 1904, vii. 474. 

161 Sachmunde, Ueber vereinzelt auftretende Halluzinationen bei Epileptiken. 
Monat. f. Psych, u. Nerv., 1904, xv. 434. 

162 Voisin, op. cit. 

153 H. C. Wood (Medical News, 1894, p. 707) has reported a case of epilepsy with 
migrainous symptoms. Such a form might appropriately be called migrainoid epi- 
lepsy. Hughlings-Jackson (Lancet, 1875, vol. ii. p. 244) spoke of migraine as 
47 



738 THE EYE AND NERVOUS SYSTEM 



THE BLINDNESS DUE TO URiEMIA AND OTHER 
BLOOD-STATES. 

Under this heading will be described those attacks of blindness, 
usually sudden and sometimes transient, which are observed occasion- 
ally in ursemic patients, also in anaemic and other blood-states, and 
likewise those due to embolus of the retinal artery. This chapter, how- 
ever, does not include in its scope an account of the permanent changes 
in the fundus oculi due to Bright' s disease or to other organic diseases 
in the optic nerves and brain. 164 

Ursemic Blindness. — Sudden blindness sometimes occurs in ursemic 
patients. It is usually seen in cases of kidney disease that are well 
advanced, although cases have been reported in which the amaurosis 
was an early symptom. But, whether early or late, the amaurosis is 
usually a sign that the renal disease is severe, and the inference is that 
the system is profoundly poisoned. The characteristics of this blind- 
ness are its sudden onset, its completeness, the preservation of the reac- 
tions of the pupil, the sudden disappearance of the blindness when the 
blood-state is relieved by active purgation or sweating, and the absence 
of ophthalmoscopic changes, excepting such as may previously have 
existed. 165 

The other symptoms associated with this ursemic blindness may 
or may not be marked. In many cases there are no prominent brain 
symptoms of ursemia, no coma, no convulsions, or any special signs of 
ursemic encephalopathy. In other cases the visual phenomena precede 
a more active and extensive attack of ursemic-poisoning. There may 
be some headache and mental confusion, which pass away with the 
blindness under active treatment, or the case may advance to convul- 
sions and coma. Most of the cases put on record have been those in 
which the blindness was the prominent or only symptom. It is proba- 
ble that some affection of vision precedes most attacks of ursemic con- 
vulsions and coma, but is overlooked in the gravity of the succeeding 
symptoms. 

There has been much speculation about the causes of the sudden 
and transient blindness of ursemia. Most observers are agreed that 
there are no ophthalmoscopic changes to account for it, or that such 

" sensory epilepsy," and he had a rather fanciful theory that it is due to a " dis- 
charging lesion of the occipital lobe." There is no scientific warrant for such a 
pathology or such a terminology. 

164 Antonalli, L'Amblyopie Transitoire, in Arch, de Neurolog., 1892, pp. 202, 423. 

165 Gowers, Manual of Medical Ophthalmoscopy, 1904. 



UREMIC BLINDNESS 739 

changes, if present, are slight. Gowers says that slight, transient oedema 
of the papilla has been noted to coincide with the symptom and to 
pass away with it. This is also noted by Orters. 166 In some cases, 
however, even this oedema of the nerve-head has not been seen. 167 Con- 
sidering the frequency of retinal changes in chronic Bright's disease, 
it is rather remarkable that no alterations are reported in so many 
cases of sudden blindness. Gowers does, indeed, say that permanent 
retinal changes are present in some cases, but that these, while, of 
course, they persist after the attack, are not changed by it. It might 
well be difficult to detect transient changes under such circumstances. 

It is natural to fall back upon the theory of an acute poisoning of 
the nerve fibres, without visible organic changes. This is a reasonable 
theory, in accord with the condition of the patient, and in accord also 
with what we know about acute poisoning of nervous tissue. A toxic 
substance in the blood might well affect the nutrition and function of 
the optic nerve and retina without giving signs that could always be 
recognized with the ophthalmoscope. Thus the blindness might be due 
to the overwhelming of the nerve with the ursemic poison. 168 This is 
the chemical theory, 169 according to which deleterious substances are 
circulating in the blood, though what the exact chemical composition 
of them is remains unknown. 

Another theory is the mechanical one, according to which the blind- 
ness is due to an oedema of the brain and of the optic nerve and 
retina. 170 We have already referred to the fact that oedema of the 
nerve-head has been noted by various observers. It has been argued 
by Hauenschild that oedema of the nerve-head alone could not cause 
the symptoms, for the reason that the pupillary reflexes are preserved; 
therefore, the lesion must be located back of the primary optic ganglia, 
possibly in the brain cortex. This is an ingenious argument, and not 
easily answered. Certainly, if the pupils react to light, the retinal 
cells must be capable of taking impressions and sending them on to the 
ganglia ; in other words, the functions of the optic nerves are not 
entirely gone, as the total blindness would seem to indicate. 

This mechanical theory, or theory of pressure due to oedema, is 
much the same as the vasomotor theory, which here, as elsewhere, has 
played its part. This is, however, a most unsatisfactory theory by 
which to account for anything in pathology, for although the vasomotors 

166 Dobrowolsky, Klin. Monatsbl. f. Augenheilk., March, 1881, p. 121. 

167 Hauenschild, Muench. med. Woch., 1903, No. 4. 

168 Benschoten, Providence Med. Journ., July, 1903. 
lee F rer i c hs, Arch. f. Physik. Heilk., x. 34. 
170 Traube, Schmidt's Jahrbuch., cxiv, p. 308. 



740 THE EYE AND NERVOUS SYSTEM 

are undoubtedly a means by which disease processes act, they never- 
theless must require some cause to set them in action. It is this cause, 
Avhatever it may be, acting upon the vasomotors, that is the true cause 
of any disease process, not the vasomotors themselves. A poison can 
conceivably affect the vasomotors, as can also mechanical irritation or 
a gross lesion, but nothing is explained by merely appealing to the state 
of the vasomotors so far as essential causation is concerned. Pal, 171 
discussing the pathogenesis of acute transitory amaurosis of uraemia, 
eclampsia, etc., says it is due to a transitory anaemia of the brain due 
to vasomotor change, cardiac insufficiency, etc. This seems like beg- 
ging the question. Certainly, cardiac insufficiency is not present in 
most of the cases of puerperal eclampsia, in which, just as in uraemia, 
some of these affections of sight are observed. Benschoten suggests 
that the blindness may be due to a spasm of the retinal vessels. Bull 172 
suggests that arteriosclerosis of the ophthalmic and internal carotid 
arteries may be the underlying cause of some cases of obscure blind- 
ness, especially shown by a paracentral scotoma. 

In uraemic amaurosis there is usually an obscuring of the whole field, 
and equally in both eyes. But Pick 173 reports a case. in which there 
was an homonymous hemianopsia of ursemic origin. 174 

Ursemic blindness has been observed in various forms of kidney 
disease. In Hauenschild's case there was a parenchymatous nephritis. 
The condition may occur in contracted kidney also. Roscher 175 reported 
a case in which there was a suddenly developed hemorrhagic nephritis ; 
two days later ursemic symptoms appeared, with amaurosis. In this 
case there were normal eye grounds, but the pupils were dilated and 
would not react, contrary to what is usually reported. This case was 
rapidly fatal, and Roscher believed that the immobility of the pupils 
suggested a bad prognosis, because it indicated the severity of the 
process. 

In puerperal eclampsia there are eye symptoms exactly like those 
seen in uraemia. Disturbance of vision is observed in many cases ; 
there may be amblyopia or amaurosis. The late Professor Parvin 176 



m Centblt. f. inn. Med., 1903, No. 17. 

172 Ann. Ophthal., 1904, xiii. 74. 

173 Ueber Hemianopie bei Uraemie, Munch, med. Woch., 1903, p. 2125. This 
condition was first described by Pick in 1896. A few cases have been described 
by others. 

174 See also Gill, Transient Recurrent Attacks of Lateral Hemianopsia, in 
Brit. Med. Journ., Feb. 1, 1890, p. 233. 

175 Muench. med Woch., 1903, No. 9, p. 382. 

170 Eclampsia, in Hirst's Am. Syst. of Obstetrics, vol. ii. p. 73. 



UREMIC BLINDNESS 741 

referred to a fatal case of eclampsia occurring at the fourth month of 
pregnancy in which there was almost total blindness for two days before 
the seizure. Dewees 17T many years ago said that a temporary loss of 
vision was among the premonitory symptoms of puerperal convulsions. 
Of recent years this symptom has been so generally recognized as not 
to require us to quote many authorities. Neither is this the place to 
enter upon a discussion of the question whether eclampsia is identical 
with ursemic convulsions. 178 Certainly it resembles the uraemic fit in 
many of its symptoms, among which is amaurosis. 

Anaemia from hemorrhage and other causes may produce sudden 
blindness. This blindness may be trifling or complete, transient or 
permanent, immediate or postponed, and more frequent from so-called 
spontaneous than from traumatic hemorrhage. The pupils are usually 
dilated and immobile to light. It has been observed after hemorrhage 
from the bowels, uterus, nose, as well as after venesection and trauma. 
The visual disturbance may be in the nature of a scotoma, or hemian- 
opsia, or contraction of the fields, or the blindness may be in sectors 
or irregular. 179 There may be changes in the fundus, or this may be 
normal. Benschoten says that the prognosis is good if the blindness 
is immediate, but unfavorable if it is delayed several days, because in 
the latter case it may be due to neuritis or atrophy of the optic nerve. 
He believes that the prognosis is worst in cases of hemorrhage from 
the stomach. Assicot 18 ° reports two cases of permanent blindness due 
to hemorrhage from the uterus. In transitory blindness due to anaemia 
Loewe 181 supposed that there was a spasm of the blood-vessels supplying 
the cuneus, a theory to be taken for what it is worth. Transient loss 
of sight occurs also in syncopal attacks. 

In diabetes there are sometimes observed defects of sight which are 
similar to ura?mic amaurosis. 182 Lecorche called attention to the grave 
significance of diabetic amblyopia in some cases, in which it may be 
a terminal symptom, or at least herald the end. 183 It should not be 
forgotten that cataract occurs in some cases of diabetes. 

177 Midwifery, 3d Ed., Philada., 1828, p. 451. 

178 Winckel, Obstetrics, Am. Trans., p. 587 et infra. 

179 Gowers, op. cit. 

180 Pathogenie des Amauroses Posthemorrhagiques, in Arch, de Ophth., May, 
1902. 

1S1 Ein Fall von Transitorischer Blei Amaurose, in Arch. f. Augenheilk., xlviii. 
p. 332. 

182 Gowers, op. cit. See also Heine, Deutsch. med. Woch.., 1903, p. 348; and 
Brunton, in Reynolds's Syst. of Med., vol. iii. p. 591. 

ls3 Traite du Diabete, 1876. Also Laveran et Teissier, Pathologic Medicate, 
3d ed. vol. i. p. 314. 



742 THE EYE AND NEKVOUS SYSTEM 

Jacquean has even written of amblyopia of hepatic origin. 184 Am- 
blyopia from tobacco and alcohol is usually due to a post-orbital optic 
neuritis, and is not to be described in this chapter. 185 

Finally, brief reference may be made here to embolus of the retinal 
artery as a cause of sudden blindness. This blindness may be per- 
manent or it may tend to improve. The causes are such as give rise 
to embolism in other arteries, especially diseases of the endocardium; 
and there may be also arterial sclerosis with temporary stoppage of the 
circulation. Emboli and thrombi may occur in these cases in other 
arteries in the brain. 186 In a case observed by the present writer in 
an old lady the blindness was sudden, complete, and permanent, and 
the embolus was doubtless derived from the heart, which was diseased. 
The ophthalmoscopic changes are usually well marked, and consist in 
a diminution in the size of the artery and, in time, atrophy of the disk 
and retina. 187 

184 Bull, et Mem. Soc. Franc. d'Ophthal., 1902. 

185 De Schweinitz and Edsall, Concerning a Possible Etiological Factor in 
Tobacco and Alcohol Amblyopia, etc., in Am. Journ. Med. Sci., 1903, p. 216. 

186 Posey (Transient Monocular Blindness, in Journ. Am. Med. Asso., May 
31, 1902) has described cases in which the blindness was evidently due to vascular 
defects other than embolic, as the loss of sight was only temporary. In some cases 
there was actual sclerosis. 

187 Norris, Medical Ophthalmology, in Pepper's Syst. of Med., vol. iv. pp. 740, 
741. See also, Van Duyse, Arch. d'Ophth., 1902, p. 93. 






CHAP TEE XVIII. 

NEUEOSES AND OTHEE CONDITIONS OCCASIONED BY 
EEEOES OF EEFEACTION AND IMBALANCE OF THE 
EXTEA-OCULAE MUSCLES, AND THE EFFECT OF LENSES 
AND OPEEATIVE PEOCEDUEE IN EELIEVINC THE SAME. 

Part L 

By S. D. RISLEY, M.D. 

HEADACHE; NEURALGIA. 

The design in the present chapter is to treat primarily of headache 
and its relation to eye-strain ; but it will not be found possible to avoid 
a more or less extended consideration of a group of associated phenom- 
ena composing the symptom complex known as " asthenopia." 

In the discussion of the subject it will be maintained — 

(1) That in a large number of patients anomalies of the ocular 
apparatus are the sole and sufficient cause of headache. 

(2) That these anomalies of vision may be the unsuspected cause 
and, therefore, that the absence of symptoms obviously referable to the 
eyes does not exclude them as an etiologic factor in headache and certain 
associated symptoms, — e.g., insomnia, vertigo, petit chorea in children, 
stomach derangements, and impaired general nutrition. 

(3) That the congenital origin of these ocular anomalies does not 
exclude them from participation as a factor in the sudden, or recent, 
development of symptoms coming on during, or after, middle life, after 
attacks of acute disease, or more or less acute exacerbations of some 
general dyscrasia. 

(4) That, therefore, the participation of the eyes as a probable etio- 
logic factor in headache and other neuroses can be positively excluded 
only in the absence of ocular disease, or after the most painstaking cor- 
rection of any existing error of refraction or abnormality of binocular 
balance. 

(5) That in many cases the prolonged eye-strain, consequent upon 
existing anomalies of vision, sets up pathologic conditions of the fundus 
oculi, and that these require time for recovery, therefore, immediate 
relief of symptoms is not always to be expected. 

The facts set forth briefly in the preceding propositions may be 
claimed as among the most signal achievements of modern ophthalmol- 



43 



744 THE EYE AND NERVOUS SYSTEM 

ogy. While it is true that a number of early observers had recognized 
a more or less definite association between weak eyes and pain in the 
brow, they failed to grasp the true relationship of the symptoms. 
Indeed, any historical study of the early literature of headache, or even 
of much that has been written by later authors, is disappointing when 
viewed from the standpoint of the ophthalmologist. In the general or 
specific discussion of this symptom and its etiology by some authors, 
the eyes as a probable factor are noticed in such a casual manner that 
it is obvious no adequate comprehension of the importance of eye-strain 
had been reached. To the ophthalmic surgeon this practical neglect 
of ocular affections as a cause of headache and a considerable group of 
associated nervous symptoms is unaccountable, since in the daily rou- 
tine of his work he encounters it in so large a percentage of his eye- 
strain patients. In 1899, the writer made a cursory analysis of one 
thousand consecutive eye patients, and found that upward of 50 per 
cent, of them complained of headache, and that many of them had been 
sent by their physicians in the hope that the pain in the head, which 
had proved rebellious to general treatment, might be due to some ocular 
defect. A colleague, Dr. M. W. Zimmerman, in an elaborate study 
of two thousand patients, drawn from private and hospital cases, found 
that 71.3 per cent, of them suffered from headache. In ophthalmic 
practice, therefore, the subject assumes a place of commanding 
importance. 

Until within the memory of men now living, and actively engaged in 
professional work, ophthalmic literature was no less at fault. We 
are, nevertheless, indebted to many of the early writers for painstaking 
and accurate descriptions of the symptom complex we now know as 
asthenopia. The weak eyes, which baffled all their efforts for relief, 
were associated by them with brow pain, and were carefully described 
by Taylor in 1776; by Plenck in 1792; by Scarpa in 1816, and by 
Beer in 1817 (Donders). These observers were followed, prior to 1850, 
by Lawrence, Tyrrel, Sichel, MacKenzie, and others. The variety of 
opinions expressed by these eminent men and the many designations 
devised and employed as either descriptive of the observed conditions 
or explanatory of their supposed nature or cause convict them of error. 
This is not surprising since it was not possible to form any adequate 
conception of the essential nature of the morbid conditions they observed 
until after the discovery of the hypermetropic eye, and the publication, 
in 1858, of the justly renowned physiologic researches of Donders of 
Utrecht in his classic treatise on the "Anomalies of Refraction and 
Accommodation." This remarkable book shed a flood of light upon 



LEUKOSES OCCASIONED BY EYE-STRAIN" 745 

many ocular problems, and laid the foundation for a most important 
part of the beautiful structure which modern ophthalmology has reared. 

This great master does not, however, seem to have comprehended the 
full clinical significance of the truths he had discovered and established. 
The ulterior results of the strain upon the accommodative function of 
the eye in overcoming the anomalies of refraction, and of the struggle 
required to maintain binocular single vision in the presence of some 
abnormality of the extraocular muscular apparatus, have for the most 
part been disclosed by his followers. The credit for recognizing the great 
significance of eye-strain in producing both the pathologic states of the 
fundus oculi, and an associated group of general reflex symptoms, often 
simulating organic disease, is largely due to American observers. Dr. 
William Thomson, in 1879, 1 in a paper on "Astigmatism as a Cause 
for Persistent Headache and Other Nervous Symptoms, " states that 
more than ten years before he became aware that persons who con- 
sulted him for defective sight presented symptoms which he enumerated 
as pain in the brow, temples, and occiput, a sense of fulness in the head 
amounting to vertigo and nausea, insomnia, loss of appetite, fear of 
impending apoplexy or epilepsy, general nervous prostration, choreic 
twitching of the muscles of the head and face, all of which he had 
seen relieved by correcting their astigmatic eyes. Other ophthalmic 
surgeons had also witnessed the marked relief of headache, insomnia, 
and other reflex symptoms, following the correction of optical defects 
by glasses. 

Dr. S. Weir Mitchell's attention was called to these cases, and to 
him is due the credit of impressing upon the general profession, in two 
forcible articles, 2 the grave significance of eye-strain. Ever since atten- 
tion was thus fixed upon the subject, in Philadelphia at least, no study 
of a rebellious case of headache or of obscure nervous disease has ever 
been considered complete until a careful examination of the eyes had 
excluded them as a possible cause of the disturbance. 

It does not fall within the scope of this chapter to consider in detail 
the nature and variety of the ocular anomalies which may cause an 
undue strain or tension upon the visual organs, but it is important to 
have a definite conception of the normal or essential requirements for 
comfortable binocular vision. That is to say, a physiologic standard 
to which anomalous states may be referred for comparison. 

This standard will be found in two healthy emmetropic eyes with 



1 The Medical News and Library. 

2 Medical and Surgical Reporter, 1874; American Journal of the Medical 
Sciences, 1876. 



746 THE EYE AND NERVOUS SYSTEM 

normal sharpness of vision, a physiologic range of accommodation and 
a proper binocular balance ; that is to say, emmetropia, or the ability 
to focus parallel rays of light on the fovea without effort of the accom- 
modation, and with V=6/vi for each eye ; a range of accommodation 
corresponding to the age of the individual, and the power to converge 
both eyes, equally and normally to the physiologic near-point, on the 
same horizontal plane. When these conditions obtain, a clear image 
of the approaching object is maintained upon the fovea of each eye 
from infinity to approximately the binocular near-point, without con- 
scious effort. In point of fact this ideal of requirement is compara- 
tively rarely observed; nevertheless the eyes which most nearly 
approach it enjoy the highest degree of comfort and meet the visual 
requirements of civilized life with a minimum of pain and peril. 
Indeed, under these harmonious relations it is extremely rare to find any 
ocular disturbance, even under the exacting demands of modern life. 

Disturbance of function, therefore, in the class of patients under 
discussion is ordinarily produced by some departure from this standard. 

Any thoughtful analysis of the standard as defined in the preceding 
paragraph will detect its great complexity. To secure emmetropia, two 
model eyes are required. To enjoy comfortable binocular single vision 
the complex system of extraocular muscles must be normally developed, 
have a normal innervation and a normal origin at the apex of a model 
bony orbit, and a proper attachment to the globe of each eye. The 
accommodative function of each eye must be equal and normal, and 
must act harmoniously under the same innervation impulse which con- 
trols the convergence of the two eyes to the finite point under observa- 
tion. 

That the harmony of all parts of this intricate apparatus is liable 
to frequent disturbance should not cause surprise. Putting aside the 
liability to impaired function from disease of one or more of its parts, 
which does not fall under discussion in this chapter, it remains to con- 
sider the fact that the various elements entering into this complex 
visual apparatus are subject to anatomical variations, — e.g., anomalies 
in the form of the eyeball, or anomalous distribution in the system of 
extra-ocular muscles, or less gross defects of development in the muscles 
or their nerve supply, any one or all of which would of necessity cause 
impairment of binocular vision or cause undue strain for its main- 
tenance. 

That such anomalous conditions are liable to befall the eyes is sug- 
gested by the great frequency of deformity in other portions of the 
body. That the eye is peculiarly liable would seem to be indicated by 



NEUROSES OCCASIONED BY EYE-STRAIN 



747 



the frequent distortions of the human skull, as shown by 
study of a hatter's forms, many of which are so ill shaped 
grotesque when compared with the diameters of a model 



any cursory 
as to appear 
skull. * One 

need not go far to verify the fact that these deformities of the skull 
cause, in many persons, variations in the size and form of the bony 

of the devel- 
and protect. 



orbits, and this leads to the more or less serious deformity 
oping eyeballs, which the orbits are designed to inclose 





Fig. 1.— Diagrams showing types of conformation of skulls in subjects whose eyes were highly astigmatic, 
with asymmetrical meridians, associated with binocular imbalance. 

It is not unreasonable to expect that, during development in an anoma- 
lously shaped orbit, not only certain modifications will occur in the 
shape of the eyeball itself, which would change its diameters and alter 
the radii of curvature of the cornea, but would lead also to changes in 
the length and line of direction of the optic nerves, and the origin and 
attachments, the length and direction of the ocular muscles, — condi- 
tions one or all of which would lead to more or less marked disturbance 
of the refraction and motility of the eye, and in many complex ways 
modify the conditions of binocular vision rendering it either difficult 
or impossible. (Compare Chapter XIV and XXIII.) 

*" Abnormalities of Ocular Balance," by S. D. Rislev, M.D., University Med. 
Mag., Jan. 1895. 



748 THE EYE AND NEKVOUS SYSTEM 

When we add to these congenital defects of structure and relation 
the ravages of disease, either affecting the eye itself, or its essential 
anatomical connections, it is obvious that in the elucidation of the anom- 
alies of binocular vision we have placed before us many obscure and 
complex problems which lie at the foundation of eye-strain and its 
attending group of symptoms. 

These theoretical considerations find ample demonstration in the 
daily routine work of the ophthalmic surgeon and also in the extensive 
statistics collated in the schools. * In these statistical studies it has been 
shown that the standard conditions for comfortable vision are compara- 
tively rare; hypermetropia, astigmatism, or myopia being present in 
varying degrees in the large majority of children's eyes; the baneful 
significance of these defects being shown by the associated asthenopia, 
that is to say, weak eyes, impaired function, inflammation of the margin 
of the lids, headache, and a wide complex of symptoms affecting the 
general health and rendering school life, for many children, both 
burdensome and perilous. 

The great conservatism so often displayed by nature in adapting 
the means at hand to the required end doubtless frequently overcomes 
in great measure the baneful influence which these anomalous condi- 
tions would otherwise exert over binocular vision. Eor example, the 
growing child under the demands of a steadily increasing experience 
gradually moulds, during the plastic years of growth, the existing con- 
ditions to meet more or less successfully the felt requirements for accu- 
rate vision, and victory is won in behalf of single binocular vision 
where defeat would have seemed inevitable. Unfortunately when exist- 
ing defects are great, the final outcome is frequently a failure, or at best 
but partially successful, and the child passes into adolescence or adult 
life with defective sight, and a heritage which, if unrelieved, must be a 
handicap to any ambitious career. It is the resulting conditions which 
are before us for study, since they furnish one of the most prolific 
causes of headache. 

The errors of refraction as found in practice present a great variety 
of conditions, but group themselves into certain types or classes : 1. 
Simple hypermetropia of equal degree in both eyes. 2. Hypermetropic 
astigmatism, simple and compound, with the most favorable — i.e., least 
defective — meridian of the cornea vertical, or, if inclined from the 
vertical, the inclination is symmetrical for the two eyes. 3. Hyperme- 
tropic astigmatism, simple or compound, with asymmetrical meridians, 



* Article, " School Hygiene." A System of Diseases of the Eye, edited by Norria 
and Oliver, vol. ii. 






NEUROSES OCCASIONED BY EYE-STRAIN 749 

— i.e., the meridian of least defect is vertical in one eye and inclined in 
the other or irregularly placed in a great variety of ways. 4. One 
eye is astigmatic, the other emmetropic or has a simple hypermetropia, 
or both are hypermetropic, but in different degree (anisometropia), or 
both are myopic with or without astigmatism. 5. One eye is hyperme- 
tropic with or without astigmatism, the other is myopic with or with- 
out astigmatism (antemetropia). 6. Both eyes are myopic with or 
without astigmatism, which, as in hypermetropic refraction, may be 
symmetrical or not, or present in different degrees between the two eyes. 
7. In both hypermetropic or myopic eyes with astigmatism the meridian 
of least defect may be " against the rule," — i.e., horizontal instead of 
vertical, symmetrically or asymmetrically inclined from the horizontal. 

When compared to the emmetropic or model eye all of these com- 
plex conditions are to be regarded as defects. They are essentially ana- 
tomical anomalies. When viewed from the standpoint of physiologic 
optics they constitute the errors of refraction in the eye. 

The emmetropic eye has an average length of about 22 millimetres 
and its dioptric system will focus parallel rays of light on the retina, 
at the fovea, without any accommodative effort ; that is to say, its far- 
point is infinity, its principal focal point lies on the retina. Such an 
eye has a range of accurate vision from infinity to the physiologic near- 
point. The near-point gradually recedes from the eye until at forty-two 
to forty-five years of age presbyopia is reached. Any departure from 
this ideal state of refraction must sooner or later introduce disturbance 
of function. In the routine of practice it simplifies the theoretical con- 
siderations to reduce the anomalous states of refraction, as enumerated 
above, to the ideal standard (emmetropia) by means of carefully selected 
glasses, which, when placed before the eyes, neutralize the defective con- 
ditions. To accomplish this would seem at first sight a simple problem 
in mathematical optics, but the human eye has been provided with the 
power of accommodation through the agency of which it is able to 
change the focus of its dioptric system. This power makes it possible 
even in the presence of anomalies of refraction, to so change its dioptric 
system as to focus more or less accurately the image of surrounding 
objects upon the retina. This power resides in the ciliary muscle and 
is exercised involuntarily. It follows that in any effort to measure the 
static refraction of the eye, this ever changing power of accommodation 
becomes an unknown quantity and must of necessity vitiate the results 
obtained. (Compare Chapter II.) 

It is not difficult to detect the presence of the grosser forms of these 
congenital defects, but they do not, as a rule, comprise the most im- 



750 THE EYE AND NERVOUS SYSTEM 

portant group in their relation to headache and reflex symptoms. Where 
binocular vision is impossible because of the gross nature of the mus- 
cular anomaly, or where the existing error of refraction is too great 
or of such a character as not to be overcome by any muscular effort, the 
afflicted individual perforce resigns to the inevitable, gives up the 
struggle, and is usually free from pain. It is quite different, however, 
when the existing error is of such a nature that the efforts of the indi- 
vidual can overcome it in the interests of clear vision. It is this effort 
which constitutes the eye-strain and is the cause of intra-ocular conges- 
tion, weak eyes, and pain, — in a word, of asthenopia. 

In the case of grosser defects of vision diagnosis is not difficult, 
since they are accompanied by impaired vision or by a manifest ten- 
dency to double vision. Obscurity exists only in the case of defects 
of minor degree, or when the experience and habit of a lifetime has 
taught the individual the trick of rendering the congenital ocular defect 
latent; that is to say, of covering it up by a tonic muscular tension. 
In such cases the vision with both eyes or for each may be normal, or 
approximately so; the range of accommodation and binocular balance 
may be but little if at all deranged, the existing congenital abnormality 
in this group of cases revealing itself only to careful analysis by the 
skilled observer. It becomes necessary in all such patients to tem- 
porarily suspend the action of the ciliary muscle before any trustworthy 
determination can be made of the static refraction of the eye. 

This is accomplished through the instillation of the so-called mydri- 
atics, or cyclophlegics. Notwithstanding the now well-known fact of the 
very high percentage of defective eyes and the very generally recognized 
relation between these defects and headache, the discussion of the subject 
should be approached with great conservatism. 

That eye-strain and certain diseases of the eye — e.g., chorioiditis — 
are in many persons the sole and sufficient cause for headache can no 
longer be questioned in the light of experience, since the ophthalmolo- 
gist is a daily witness of the complete relief from the malady afforded 
by the correction of errors of refraction and abnormalities in the ocular 
muscles or by the cure of intra-ocular disease. Experience, however, 
has shown that, in other groups of patients suffering from headache, 
there may be no relation between existing ocular defects and the pain 
in the head, since the most careful correction of the existing anomalies 
affords no relief. Some examples of migraine belong to this class. All 
headaches are not due to eye-strain. It is not a very unusual experi- 
ence to remove the eye-strain by glasses to the great relief of ocular 
symptoms, without any appreciable result in relieving the headache; 



LEUKOSES OCCASIONED BY EYE-STRAIN 751 

or, certain kinds of pain in the head, in any given patient, may be 
relieved and others still persist. 

It must be borne in mind also that many patients suffer greatly 
from impaired vision, chronically inflamed and painful eyes, who never 
have had a headache or other reflex symptoms. It may be stated as an 
ophthalmic aphorism that when eye-strain manifests itself by red lids, 
recurring styes and symptomatic conjunctivitis that such persons com- 
paratively rarely suffer from headache, while on the other hand the 
patients who suffer from reflex symptoms as the result of ocular defects 
may exhibit no local external inflammatory or irritative manifestations. 
There are numerous exceptions, however, to this statement. Then, too, 
the subject grows in complexity, from the standpoint of diagnosis, 
because of the fact that in two patients suffering from similar or 
identical ocular defects, one may have no symptoms other than weak 
eyes and a blurring page after prolonged effort, while the other is con- 
stantly in the slough of semi-invalidism from pain in the head and other 
reflex symptoms which, from exposure to light or the persistent use of 
the eyes, or from the general fatigue following some unusual exertion, 
culminate in more or less frequent and violent explosions of so-called 
" sick or nervous headache," and both of whom find complete and last- 
ing relief from glasses so nearly alike that they might be used inter- 
changeably. In a third case, with a similar ocular defect, there are no 
symptoms either reflex or local, the strain being borne with impunity. 
While then it is unquestionably true that in many instances the exist- 
ing ocular defects are the sole and sufficient cause for their headache, 
the great difference in the effects of eye-strain, upon different individ- 
uals, in combating the untoward conditions presented by these con- 
genital anomalies make it necessary to look beyond the eye for some 
adequate explanation. Sufficient cause for the phenomena may often 
be found in the difference of vocation, environment or temperament. 
Eor example, the persistent use of the eyes in one case may be unnec- 
essary and in the other compulsory. One may be in good general health, 
the other a victim of hereditary or other neuroses, or some complicating 
diathesis which impairs the nutrition and introduces a tendency to 
pain and local pathologic states. Ambition to excel, or the sanguine 
temperament, may drive one into constant activity in spite of the pain 
occasioned by the use of the eyes, while the other rejects the goad of 
ambition unwilling to secure success or to court fame at the sacrifice of 
pain. Then, too, in the presence of ocular conditions causing eye-strain, 
other things being equal, the neurotic individual is more likely to suffer 
from headache and associated phenomena than those who cannot be 



752 THE EYE AND JSTEKVOUS SYSTEM 

designated as nervous people. While, therefore, the general conditions 
of health or temperament or the necessity for constant use of the eyes 
may in some sense be regarded as the cause of the headache, the primary 
factor in its etiology is the eye-strain, and the pain will not be pre- 
vented until the exciting cause is removed, although it may be partially 
relieved or modified by improvement in general conditions. 

An extremely interesting group of patients are those who suffer from 
sick or nervous headache and general nervous symptoms, — e.g., nausea, 
insomnia, irritability of temper, facial chorea in children, etc., and are 
completely and permanently relieved of their symptom complex by the 
correction of some ocular defect which throughout life has been the 
unsuspected cause of their suffering. 

Many patients who suffer severely from headache demonstrably due 
to ocular defects or disease, are often quite free from local symptoms. 
If ocular symptoms are present during acute exacerbations of headache, 
they occur pari passu, or come on after the reflexes, and are regarded 
by the patient as due to headache. Patients often claim that their 
" eyes are weak because they have a bad head" and submit to examina- 
tion of the eyes reluctantly and only on the advice of their physician, 
deeming it unnecessary since there are no obvious eye symptoms and 
therefore apparently nothing wrong with their eyes. In these patients 
the location of pain is often suggestive of the ocular origin of the symp- 
toms. Vertex pain is comparatively rarely due to eye-strain. In 
optic-nerve disease, as in post-ocular neuritis and atrophy, there is in 
many cases pain in the front part of the head on top, but it is not a 
vertex pain. The pain when associated with the class of eye affections 
under discussion occurs in the brow, occiput or nape of the neck, back 
of the eyes and shooting thence to the occiput, as a hemicrania, or in 
the temples. In point of frequency the pain occurs probably in the 
order named. Simple eye-strain due to some error of refraction, — e.g., 
hypermetropic astigmatism, particularly in children, — will usually give 
rise to brow pain. If the refraction error is associated with an imbal- 
ance of the ocular muscles so that constant muscular strain is required to 
maintain single vision, as in exophoria, a tendency for the eyes to 
diverge ; or in esophoria, a tendency to converge ; or in hyperphoria, a 
tendency of one eye to turn upward, there will usually be occipital pain 
also. If at the same time marked turgidity or inflammation of the 
chorioid, retinal irritation or disease of the retina and chorioid in the 
region of the fovea have been set up as the immediate result of the 
constant struggle for accurate vision, in the majority of patients there 
will be a tense post-ocular pain which shoots to the occiput, nape of 



LEUKOSES OCCASIONED BY EYE-STRAIN" 753 

the neck, or even down the spine, not infrequently radiating to the 
shoulders and arms. In two notable instances which caused great 
anxiety, one a busy attorney, the other an eminent physician, the writer 
witnessed complete relief from pain in the region of the heart, which 
shot backward to the left scapula, follow the correction of an astigma- 
tism against the rule in the one, and correction of hyperphoria by 
tenotomy in the other. 

Many of these patients, although unwilling to ascribe their suffer- 
ing primarily to their eyes, nevertheless recognize the harmful influ- 
ence of exposure to strong light, or, that exacerbations of pain come on 
after extraordinary use of the eyes, but others never do and ascribe 
their eye symptoms to " biliousness," or to overfatigue, hunger, and 
many other things. 

Others regard their recurring sick headaches as hereditary and relate 
that one or both parents and other members of the family are victims 
to the same malady. They think, therefore, that they are born to a 
heritage of pain from which it is useless to seek permanent relief. I 
have seen many of these lifelong sufferers from recurring sick headache 
entirely and permanently relieved by the correction of their faulty ocu- 
lar conditions. It is doubtless true that the neurotic tendency may be a 
family trait, but it is equally true, even more probable, that the ana- 
tomic defects already noticed, upon which the errors of refraction or 
the ocular imbalance depend, are hereditary. The regularity with which 
defective eyes, — e.g., hypermetropia with astigmatism, — are handed 
down from parent to child is one of the most striking facts in ophthal- 
mology. It should be noted in this connection how frequently it is to the 
form of the skull that the characteristic family likeness is largely due. 
It is highly probable that the defective form of the eyeball which pro- 
duces the ametropia, and the faulty attachment of the muscles to the 
globe which causes the ocular imbalance, are the direct results of the 
defects in the shape of the bony orbit, and this in turn is due to distor- 
tion of the skull. It follows, therefore, that the possibility of hereditary 
anatomical malformations should be excluded in every case before 
accepting the theory of hereditary headache. (See Page 954.) 

There is much variety of manifestation in different patients even 
in the presence of similar ocular defects. Many suffer almost con- 
stantly from brow pain and pain in the occiput. It is quite common for 
patients afflicted with muscular anomalies to have their rest disturbed at 
night by a dull, dragging pain in the back of the head and neck. Many 
patients complain that they are often awakened in the night by this dis- 
tressing symptom and get relief only by shifting the position of the 
48 



754 THE EYE AND JSTEKVOUS SYSTEM 

head. One patient remarked that he always awoke in the morning 
feeling as though he had had a billet of wood for a pillow. He had 
in addition to his astigmatic eyes a hyperphoria and was not relieved 
of his nocturnal headache and insomnia until vertical balance between 
the eyes was restored by tenotomy, notwithstanding the fact that his 
weak eyes and headache had been relieved during his waking hours by 
glasses containing a prism which corrected the hyperphoria. 

The complaints of a busy physician who consulted the writer in the 
hope that his eyes might afford some clue to the origin of his symptoms 
furnish a vivid illustration of the unsuspected participation of the eyes. 
He suffered from almost constant occipital pain, considered himself a 
chronic dyspeptic, was in despair over the insomnia which for many 
years had been his constant terror. He had consulted many colleagues 
in different cities without lasting relief, and finally was advised to aban- 
don his work for a year and take a long sea voyage on a slow sailing 
merchantman. It was on his return from this trip, which had con- 
sumed more than six months, that he consulted me. He had left home 
" a nervous wreck," had returned much improved in many ways, but no 
sooner had he entered upon his work as a rhinologist than he suffered a 
speedy return of all his symptoms. He enjoyed normal acuity of vision 
in each eye, and had no subjective eye symptoms. The ophthalmoscope 
revealed marked retinal irritation, however, the fundus being uniformly 
red and fluffy. There was a low grade of heteronymous astigmatism, a 
tendency for the two eyes to converge, and a right hyperphoria of 3°. 
The refraction error was carefully corrected under prolonged mydriasis, 
and subsequently the hyperphoria relieved by tenotomy. Very little 
relief followed the use of the correcting glasses, but as soon as the 
vertical deviation between the eyes was relieved by operation his symp- 
toms promptly vanished, not only the headache and insomnia, but the 
dyspepsia. He ate with impunity, the morning following the operation, 
" a hearty breakfast of sausage, buckwheat cakes, and coffee," and con- 
tinued for years to eat, without subsequent harm, anything he wished. 
The general health improved and he has continued his work with satis- 
faction. 

Many persons, although rarely free from dull pain in the head, 
suffer more or less acute exacerbations after exposure to light, from 
prolonged use of the eyes at near-work, after an evening spent in the 
theatre, or by general fatigue, but their symptoms are never violent. 
Others, while like these, are rarely free from pain, are subject to stead- 
ily progressive aggravation of all the symptoms until they culminate in 
violent attacks which drive them to bed in a darkened room, from which 



LEUKOSES OCCASIONED BY EYE-STEAI1S T 755 

they emerge after twelve or twenty-four hours with a dull sore head, and 
weak eyes, which are tender to palpation and unduly sensitive to light. 

The explanation for the advancing symptoms up to the point of 
crisis is found, not only in the muscular fatigue and exhaustion of the 
nerve-centres, but in great measure is due to the steadily increasing 
hyperemia and turgescence of the uveal tract of the eyes coming on 
under the strain of near-work or by exposure to light. It is an interest- 
ing fact that the acute symptoms often present themselves many hours 
after the culminating cause. For example, an evening at the opera will 
be followed by the acute attack the following morning after a restless 
or sleepless night, the insomnia being due to the ocular turgescence. 
The relief comes after the subsidence of the ocular congestion, which 
results from the confinement in a darkened room, the emesis, the 
administration of a saline cathartic, and the cold compresses over eye 
and brow, which are the forms of treatment ordinarily adopted by the 
patient, and which experience has taught are the most efficient means 
of relief. They return to their usual vocation only to pass through the 
inevitable cycle of suffering. Many patients expect these attacks of 
headache once a week at least, and return from a journey or shopping 
expedition in the full expectation of spending the next twenty-four 
hours in bed. 

A striking illustration of this group of patients was afforded by 
a young lady, a guest in the writer's house. She arrived worn out by 
a long journey. The following morning she was unable to appear 
at breakfast, and was found in the throes of a violent sick headache. 
The eyes were red, the lids swollen, she had photopsies which she de- 
scribed as wheels of fire and circling balls of red light. The pain was 
more severe in the right side of the head. She related that she had 
suffered these attacks two or three times every month since adolescence ; 
that when the attack was imminent she usually awoke in the morning 
with dim vision, scintillating flashes of light and zigzag " wall of troy" 
lights in the periphery of the field of vision, which at times would 
present all the colors of the rainbow. These would repeatedly narrow 
down to near the centre of the field, then widen out into broader cir- 
cles, grow fainter and finally disappear, leaving her with obscured cen- 
tral vision, objects being seen as through a veil. This obscuration would 
last but a brief time, but was always followed by nausea and vomiting, 
and the onset of violent headache, usually a right homier ania at first, 
which later spread to the entire head. Every noise or ray of light added 
to her suffering, the slamming of a door being especially excruciating, 
as it seemed to her to shake the entire house. The attacks usually 



756 THE EYE AND NERVOUS SYSTEM 

forced her to remain in bed for the entire day, and her " head felt tired 
and tender" for a day and often two days after the subsidence of the 
acute symptoms. Her mother and an older brother had suffered in the 
same manner since her earliest recollection. 

A solution of atropia sulphate was instilled immediately into both 
eyes and repeated at brief intervals. All other means of relief were 
omitted. In an hour the pain subsided, and in two hours she was able 
to come down stairs to my consulting room. I there found a hyperme- 
tropic astigmatism, not in high degree, but with the axis of the required 
correcting cylinder against the rule in the left eye, and diagonal in the 
right. There were absorption changes in the fundus of both eyes, and 
macular retinochorioiditis in the right, a fact which, together with the 
diagonal corneal meridians, explained the right hemicrania. After a 
few days of treatment she received a pair of correcting glasses. These 
she has worn for twenty years with no recurrence of her attacks of 
" migraine ;" she has suffered rarely from a dull headache, but then only 
after special stress of circumstances or when her " glasses were fitting 
badly." 

Another interesting example of this group of cases was furnished 
by a lady aged 49 years, who wrote from a distant city for an appoint- 
ment. She desired a consultation at her hotel, as she wrote that she 
would be suffering too severely from " sick headache" after her journey 
to be able to leave her bed for a day or possibly two days thereafter. 
When the writer called at the appointed time he found her, as she had 
predicted, in the throes of a violent headache ; nervous, exhausted, suf- 
fering from nausea and fruitless attempts at emesis. A mydriatic was 
immediately instilled, and cold compresses applied to the closed lids, 
and potassium bromide administered. The patient was completely 
relieved in an hour, rose, ate a hearty meal, and the following morning 
was able to go to the office of the writer. Here it was ascertained that 
she had a high grade of hypermetropic astigmatism. After this had 
been corrected by glasses she was permitted to return to her home. Upon 
her arrival she wrote that for the first time in her experience she had 
been able to enjoy a railroad journey and had reached her home in per- 
fect comfort. Ocular defects are often the cause of headache after a 
journey by rail, in a carriage, or after shopping or mingling with 
crowds of people. • 

Closely allied to this group of patients are those who claim to be 
nervous and. suffer from what they call nervous headache. They are 
rarely nauseated, but have violent pains with muscular twitchings and 
hysterical crises. They usually seek for relief in opiates and analgesics. 



LEUKOSES OCCASIONED BY EYE-STRAIN 757 

Such patients are generally women, but the writer recalls the case of a 
merchant, the father of a numerous family of children, and the head of 
a large mercantile house, who had high hypermetropic astigmatism and 
right hyperphoria. The right side of the face was smaller than the left, 
the right zygoma being flat, while the left formed an arch. The hyper- 
metropia was higher in the right eye than in the left, and the principal 
meridians of the cornea were inclined 45° from the horizontal plane. 
The refraction defect had been partially corrected by glasses, but the 
ocular imbalance neglected. Suddenly, while at his place of business, 
he would have some obscuration of vision accompanied by shimmering, 
rainbow-colored phosphenes in the periphery of his field of vision. 
Taught by many experiences he would hastily summon a cab to take 
him to his home, for he recognized the phosphenes as an invariable 
prodrome of an attack of severe headache. His wife described to me 
one of these exacerbations. 

Arrived at home, his fortitude entirely exhausted, he would ring 
the doorbell violently, enter the house holding his head between his 
hands, and often unable to reach his bedroom, would throw himself 
upon the hall or parlor floor in despair, and entirely beside himself in 
an agony of suffering, burst into a flood of tears, roar with pain, making 
the most violent manifestations, alarming his wife, children, and ser- 
vants, until the doctor came and relieved him by a hypodermic injec- 
tion of morphia. These violent attacks were relieved by careful correc- 
tion of his refraction error and a subsequent tenotomy of the right 
superior rectus muscle; but he still suffers from a dull occipital pain 
after a " hard day." Since these attacks often came on after some 
fancied indulgence at the table, or after an evening spent at the theatre 
followed by a supper, he ascribed them to deranged stomach and liver, 
and was in consequence a victim to the cathartic habit. 

These violent onsets of pain in the head are occasionally associated 
with more or less violent storms of the nervous system, hysterical crises, 
cataleptoid seizures, and in one case which came under my eye the 
attacks were followed by what had at least been diagnosticated by an 
eminent authority as epilepsy. 

A man thirty-five years of age had for several years been under 
treatment for epilepsy, but suffered almost constantly from headache, 
which culminated in paroxysms of pain preceded by phosphenes, which 
had come to be regarded as the aura of his epileptic seizures. lie was 
sent to me for blurring page, and in the hope that his headache might 
be relieved by glasses. I found a moderate degree of refraction error, 
corrected by 4"±-50 s +-50 c a x. 1S0° in each eye, and a marked abso- 
lute insufficiency of the internal recti. He received correcting glasses 



758 THE EYE AND NERVOUS SYSTEM 

for constant wear and a second pair combined with prisms, base in, for 
all continuous near-work. I did not hear from him again for eight 
years, when he returned from his distant home for some change in his 
glasses, and much to my surprise said that he had not had a single 
return of his headache or convulsions after the treatment of his eyes. 
He then described to me his former attacks. Hurrying to his home 
with the first appearance of the phosphenes, he would soon be in the 
throes of a violent headache. The paroxysms would culminate in loss 
of consciousness, general convulsions, frothing at the mouth and wound- 
ing of the tongue, after which he would fall into a profound sleep from 
which he could not be awakened, often lasting for several hours. 

Another striking instance of headache associated with nervous symp- 
toms was a tall, thin, pallid, nervous lad, with red hair, age 17. He 
was the son of a busy attorney who was overworked and in feeble health, 
and a nervous, hysterical mother who had mixed astigmatism. The 
father and a brother had high myopic astigmatism and chorioiditis. 
The boy was brought because of his weak eyes and severe headaches, 
often attended with nausea and vertigo. The day before his visit to 
me, the father had found him in an unconscious condition in an arm- 
chair in the library before breakfast, with his legs and arms extended 
and rigid, his head thrown back, the eyes wide open, and the morning 
newspaper fallen from his hands. While examining. the eyes I had an 
opportunity to witness one of these cataleptic seizures, which came on 
while attempting to determine the range of accommodation. He had 
a high degree of mixed astigmatism and insufficiency of the interni. 
There was marked chorioiditis and the eyeballs were stretching; that 
is to say, the refraction was passing from hypermetropia into myopia 
through the turnstile of astigmatism. His eyes were subjected to pro- 
longed treatment and finally corrected by glasses resulting in relief so 
nearly complete that he finished his college course and became a success- 
ful civil engineer. There can be but little doubt as to the relation of 
cause and effect between his ocular conditions and the storm in his ner- 
vous system. Although he received alteratives internally, and his 
general regimen was carefully regulated, all this had been done before 
by a wise and experienced family physician who still conducted the 
general treatment. 

The group of patients illustrated by the foregoing examples, who 
had all been lifelong sufferers from headache and other neuroses, aggra- 
vated by any occupation involving the use of the eyes, are those had in 
mind by Gould in his Biographic Clinics, and render highly probable 
the truth of his contention, at least in the examples selected by him from 
famous literary folk, by way of illustration of his thesis. They all may 



NEUKOSES OCCASIONED BY EYE-STKAUST 759 

without difficulty, so far as the symptomatology is concerned, be repro- 
duced again and again from the case books of every ophthalmologist. 

A class of patients, some of whom present great difficulty in diagno- 
sis, are those who develop headache during and after middle life. The 
pain is periorbital, post-ocular, or occipital, and may present any of the 
characteristic features already described in the preceding groups. The 
pain may be dull and constant, more or less aggravated by using the 
eyes or by exposure to light, or they may have severe periodical exacerba- 
tions of pain. Any existing refraction error or other ocular defects 
have heretofore given no evidence of their presence, or the eye may be 
approximately emmetropic and the vision normal, and the patient never- 
theless associate his pain with the use of his eyes. A careful ophthal- 
moscopic study reveals a maroon-colored fundus with pigment clouds, 
and fine granular changes in the region of the fovea and between it 
and the optic nerve. The nerve itself, while possibly clearly outlined, is 
too vascular and appears therefore too deeply colored. The history may 
be entirely negative regarding any recognized ocular disturbance in the 
past. There is a widespread feeling in the community that glasses 
should be avoided as long as possible, many persons taking pride in the 
fact that they are forty-five or even fifty years of age and have never 
worn glasses. The fact is that, given an emmetropic or model eye, the 
physiologic range of accommodation is so far diminished in most per- 
sons soon after forty years of age, that a weak convex glass for reading 
will afford both additional comfort and safety. When an extra innerva- 
tion strain must be placed upon the accommodation to secure satisfac- 
tory vision in reading, certain anomalies of convergence are produced 
which disturb the ocular balance and all of the chain of phenomena 
depending upon eye-strain follow, — e.g., headache, intra-ocular hyper- 
emia, and chronic irritation or inflammatory changes in the fundus. 
As representing this group may be cited the case of an eminent clergy- 
man aged fifty. This gentleman had resting upon him not only the 
responsibilities of a large and influential parish, but often occupied the 
lecture platform, and was a frequent contributor to religious and lit- 
erary periodicals and the author of several books. He began to suffer 
from insomnia and almost immediately became the victim of severe 
occipital pains and occasional attacks of vertigo, for which he sought 
the advice of his physician. A diagnosis of cerebral hyperemia from 
overwork was made and a grave prognosis given. He was advised to 
abandon his work for a year and seek relief in foreign travel. Quite 
disheartened, he consulted the writer and told him of his plans. His 
symptoms suggested the possibility of eye-strain, and brought out the 



760 THE EYE AKD NEKVOUS SYSTEM 

fact that lie had never worn glasses notwithstanding his age, and the 
enormous amount of literary and other work he was doing. He received 
a pair of working glasses. His insomnia and headache disappeared 
promptly, and although arrangements had been made for a long vaca- 
tion they were cancelled and he went on with his work. He was to all 
appearances in perfect health and was a noteworthy example of stalwart 
manhood. There was, therefore, no suspicion of any neurotic or other 
predisposing element entering as an etiologic factor, his symptoms 
unquestionably being the result of his neglect to neutralize by glasses 
the physiologic loss in his range of accommodation. 

Another simple explanation for the sudden onset of pain after 40 
years of age is the existence of minor errors of refraction which have 
given no recognized trouble until the diminishing range of accommoda- 
tion has arrived at a point where the accommodation strain sustains 
such a relation to its total value that it can no longer be borne with 
impunity and hence the onset of pain and its attendant phenomena. 
The only source of relief for such patients is the use of carefully selected 
glasses. These two groups of patients fortunately comprise the larger 
percentage of patients between forty and fifty years of age who seek 
the advice of the ophthalmologist for the somewhat sudden onset of 
headache. 

It must be borne in mind, however, that the loss of accommodation 
is due to a general diminution in the elasticity of the crystalline lens, a 
change probably indicative of like degeneration processes going on in 
the other tissues of the body and which culminate in old age. They 
have at least reached the age of beginning degeneration. 

In many persons certainly, the wear and tear of life begins to mani- 
fest itself between forty and fifty years of age in tissue changes, often 
pathologic in character. The commencing affections of the general vas- 
cular tree are visible not infrequently to the magnified image of the de- 
tails of the eye-ground furnished by the ophthalmoscope before any sus- 
picion of their presence is afforded by any notable general symptoms; 
but the appearances in the fundus, while they awaken the suspicion of 
the ophthalmic surgeon, and may lead to a study of general conditions, 
are not always characteristic of any specific dyscrasia and may readily 
be ascribed to the irritation and other local changes caused by pro- 
longed eye-strain, as described in the preceding paragraphs. It is there- 
fore possible to err in diagnosis at this point, since the vascular optic 
disk, the general fundus conditions, and the headache may be a part of 
the symptom complex of some serious dyscrasia which is sapping the 
vital functions. The existence, therefore, of some refraction defect, or 



LEUKOSES OCCASIONED BY EYE-STRAIN 761 

neglect of the timely use of suitable glasses for near work should not 
be too readily accepted as the sole cause of headache in patients who 
have reached middle age. 

The following case will illustrate the need for great care. 

Mr. X., aged 49 years, an artist who spent many hours daily in his 
studio, had for several years suffered more or less constant occipito- 
frontal pain with frequent paroxysms of nervous headache which forced 
him to bed. In 1902, following one of these attacks, he noticed a central 
blurred spot before the left eye. This proved to be due to a minute 
hemorrhage at the fovea. The fundus of both eyes was cinnabar red, 
and there were numerous dark pigment clouds in the periphery. The 
veins were too full and dark, the optic nerves dark red but not swollen, 
there were faint gray lines along the larger retinal arteries, and fine 
granular changes in the macular region of both eyes. He was using 
for his work weak convex spherical glasses, whereas he had in both eyes 
hypermetropic astigmatism requiring the axis of the correcting cylin- 
ders against the rule. Here was sufficient cause for his headache, and 
it is not uncommon to find similar fundus conditions due solely to the 
continued eye-strain to which his eyes had been subjected. The macular 
hemorrhage, however, and the gray outline of the arteries, which sug- 
gested endarteritis, could not be overlooked. He ascribed his trouble 
to an attempt to reproduce upon the canvas the glint from a bright 
sabre which was placed in such a position as to reflect a strong light. 
The effort had developed a severe paroxysm of headache. He consid- 
ered himself in good general health. He had never eaten meat, having 
been reared as a vegetarian. There was no discoverable arterial athe- 
roma, but he had an accentuated systole and any vigorous exertion 
caused dyspnoea of a mild type. His habits were sedentary. He often 
passed too great quantities of urine, and a specimen examined was 
negative but had a low specific gravity and a few hyaline casts. His 
health failed rapidly, an intermittent albuminuria developed, and he 
died in urgemic convulsions in a year. The acute symptoms came on 
while he was on a sketching tour in the mountains, and was ushered in 
by one of his paroxysmal headaches. The glasses he received had for 
some months modified his head symptoms, and doubtless the eye-strain 
was a factor in their etiology, but behind the local conditions lurked, 
unsuspected by himself or his family physician, the general dyscrasia 
which led to a speedily fatal termination. 

Dr. IL, aged 56, a dentist, became the victim of insomnia, dyspeptic 
symptoms and occipital headache with frequent attacks of vertigo. A 
diagnosis of " concealed gout" had been made, and his headache 



762 THE EYE AND NERVOUS SYSTEM 

ascribed to cerebral hyperemia. The general regimen bad been care- 
fully and wisely regulated, comprising a prescribed diet, horseback 
exercise, bathing, etc., together with lithia waters and drugs. In spite 
of an intelligent and rigid obedience in carrying out the prescribed 
rules laid down for his guidance, the symptoms persisted and drove 
him from his work daily with severe occipital pain. Tie was then 
advised to abandon his work and spend six months in Switzerland. 
He experienced marked relief and returned to his chair, only to suffer 
a speedy relapse. He consulted me then of his own volition about his 
eyes. He was unquestionably the subject of lithsemia, but that this 
was not the sole cause of his headache was demonstrated by a steady but 
rapid subsidence of his symptoms after the correction of an astigmatism 
which had been neglected in the glasses he had been wearing. He is 
now nearing seventy years of age and has steadily pursued the arduous 
duties of his profession without a return of his symptoms. 

It is not unusual for a patient during or after attacks of acute 
disease, — e.g., coryza, influenza, pneumonia, etc. — to become the victim 
of headache which persists and is aggravated into acute paroxysms by 
the use of the eyes. Examination reveals the fact that they are sub- 
jects of some congenital anomaly of refraction, which has for the first 
time manifested itself in the symptoms of eye-strain, under the low- 
ered physical tone produced by disease, and they are never again able 
to avoid the use of correcting glasses. 

There are numerous patients who never suffer severe pain as the 
result of their eye-strain, but are nevertheless victims of a group of 
symptoms which must be classed as ocular neuroses. Insomnia has 
been mentioned as a frequent symptom. The reverse of this is a com- 
mon complaint. Many persons become drowsy, especially in the even- 
ing, and find it quite impossible to read or do any eye work without 
falling asleep. I have many times seen this relieved by a pair of 
glasses. A constant sense of general fatigue or malaise is often caused 
by eye-strain. A busy physician came for relief for some illy defined 
ocular disturbance. He was 49 years of age and used -f-2D. for 
reading. He received for constant wear the following formula for 
glasses : 

O.D. + .62 C + 1.° ax. 30° V. = ~ 

O.S. + 1.00 C + 1.° ax. 150° V. = =4 

V 1. 

with a reading segment added of + 1.75D. 

He returned in a week to express his satisfaction and remarked that 



LEUKOSES OCCASIONED BY EYE-STKAIX 763 

he had been chronically tired for a long time ; had had his heart exam- 
ined and an analysis made of his urine, fearing the incipiency of some 
organic disease, but that all these symptoms had promptly disappeared 
under the relief from his eye-strain. 

The facial twitchings of school children, with brow pain, irritabil- 
ity of temper, uncontrollable restlessness, disturbed sleep, precarious 
appetite, and poor progress at school, all of which make them the despair 
of their mothers and teachers, have many times been relieved by a pair 
of glasses. Whether these cases can be properly classed as petit chorea 
or not, it is nevertheless certain that the eye-strain is for them " the 
thorn in the flesh" which produces a constant irritation of the nervous 
system. In another group, although the defective eyes are evidently 
an important factor in the causation of symptoms, they are obviously 
not the sole cause. The above symptom complex may be present, but 
there is in addition many evidences of a vicious metabolism. The 
eye ground is fluffy and turgid, with here and there areas of pigment 
absorption ; the veins are large and dark, the lymph channels are 
engorged, the optic disk is too red and its margins obscured ; the retro- 
tarsal folds of the conjunctiva are thickened and opaque, the caruncles 
are swollen and red, they have blepharitis and frequently recurring 
styes. A careful and painstaking study will in many cases reveal an 
irregular astigmatism as shown by the ophthalmometer or by skioscopy. 
They suffer severely from frontal headache and undue sensitiveness to 
light ; the anterior perforating blood-vessels are enlarged. Xo glass 
will afford a normal acuity of vision. The pain in the head, the restless 
sleep, the precarious appetite, the muddy skin, the many evidences of 
deranged alimentation and impaired metabolism, make it difficult to 
draw the line sharply as to the relative responsibility of local and gen- 
eral conditions. The eyes are unquestionably suffering from the strain 
at school in overcoming a congenital anomaly. The anterior segment 
of the eyeballs is stretching under the increased pressure from within, 
due to the hyperemia and turgescence of the intra-ocular tunics, and 
this increased tension is doubtless in some measure the origin of the 
brow pain and other reflex phenomena ; but it is doubtless true also 
that these local ocular conditions might not, and probably would not, 
have occurred, in many cases certainly, but for the predisposing influ- 
ence of the impairment of the general nutrition. While, therefore, it 
would be a fallacy to ascribe all of this group of conditions to eye-strain 
alone, nevertheless the correcting glasses and prolonged rest from near- 
work often prove of great relief to both eyes and head. The sleep 
improves, the nervous symptoms disappear, and in many instances the 



764 THE EYE AND NERVOUS SYSTEM 

improvement in general health is so speedy, following the treatment 
of the eyes, that the inquiry is suggested whether the impaired metab- 
olism was not in large measure due to the restless sleep, and constant 
headache due to ocular conditions. Analogous states have been 
observed as due to pharyngeal adenoids and to phymosis, so that it is 
not unreasonable to suppose that similar general conditions may be 
produced by the pain and irritation of prolonged eye-strain, the conse- 
quent fatigue of the nerve-centres involved, and the turgid states of the 
intra-ocular tunics. 

The headache and insomnia of aged people is not infrequently pro- 
duced by similar conditions of the chorioid and retina, and rapidly 
disappear under suitable local treatment addressed to the ocular condi- 
tions alone. I have seen many examples of this, some of which excite 
commiseration. The following history is illustrative : Miss McA., aged 
68 years, came for advice regarding her failing vision, inflamed eye- 
lids and a troublesome nictitation. Vision was much reduced in both 
eyes, but was improved by convex glasses. She had hypermetropic 
astigmatism in both but much higher in the left eye than in the right. 
There was no complaint of pain. The caruncles were red and swollen, 
there was long-standing blepharitis, incipient cataract, floating vitre- 
ous web, and chorioiditis with macular retinitis were present in both 
eyes. Inquiry about her general health educed the reply that all her 
life she had been a victim of sick headaches. She recalled that as a 
small child she returned from school with red eyes and headache. Bury- 
ing her head in her mother's lap she would burst into tears from the 
violent pain. These attacks had continued until fifty years of age, 
but since then, although she suffered more or less constantly from 
" neuralgia," the pain did not cause nausea or emesis as in former 
years. The neuralgia had been " better of late because of medicine 
from her physician," which proved to be Kenyon's neuralgia pills. So 
utterly was this lifetime of pain divorced in her mind from ocular par- 
ticipation that she did not think it necessary to mention it until the 
history was drawn out by inquiry. Yet the ophthalmoscope revealed the 
scars produced by years of eye-strain, which was finally culminating in 
impaired nutrition of the vitreous body and the crystalline lens. The 
neuralgia pill was omitted, homatropine instilled four times daily, and 
smoked glasses with complete rest of the eyes advised. In a few days 
the external redness and nictitation disappeared, she slept soundly, the 
appetite improved, and a sense of discomfort and tension about the 
head vanished. 

It is therefore important in both children and adults who are ob- 






LEUKOSES OCCASIONED BY EYE-STKAIN 765 

viously the subjects of impaired general nutrition and suffer from 
headache that the ill health should not be too rigorously regarded as 
the sole cause of all their symptoms. 

In a large percentage of persons whose headaches are without 
question due solely to eye-strain and its associated local conditions, the 
symptoms disappear promptly and permanently under correcting 
glasses, but in many others the result is disappointing. It has already 
been pointed out that as a result of protracted eye-strain pathologic 
states. of the fundus oculi are produced. The turgid, fluffy chorioid, 
absorption chorioidal crescents at the temporal margin of the optic 
nerves, granular changes at the fovea with impaired acuity of central 
vision are conditions witnessed daily in the consulting room as the out- 
come of constant strain required to overcome the existing congenital 
defect. The congestion of the vascular chorioid produces increased 
intra-ocular tension and pressure upon the terminals of the ciliary 
nerves, resulting in headache and the other reflex symptoms under dis- 
cussion. The tender, readily-yielding sclerotic coat of childhood often 
yields to this increased pressure from within ; the eyeballs stretch and 
pass from hypermetropic refraction into myopia. It is to these condi- 
tions that the increasing percentage of near-sight among school children 
is due, and statistics have shown that in this group is found the sufferers 
from headache, restlessness in school, blepharitis, weak eyes, and im- 
paired acuity of vision. The distention of the globe is not always uni- 
form, sometimes falling upon the posterior pole as in many cases of 
high myopia, but often affecting the anterior segment, especially in the 
ciliary region, which becomes thin, appears bluish in color, and is trav- 
ersed by the dilated anterior ciliary blood-vessels which perforate the 
globe in this locality to anastomose with the long posterior ciliary 
arteries in the ciliary region of the uveal tract. It is in these cases of 
change in the curvature of the anterior portion of the ball that varia- 
tions in the corneal astigmatism are observed. Now it is not to be 
expected that these conditions, which gravely involve the nutrition of the 
entire globe of the eye, will vanish at once under the use of correcting 
glasses. More or less protracted rest from use at a near-point, avoid- 
ance of exposure to strong light, careful attention to the general regi- 
men, and, more than all, the passage of sufficient time for recovery, are 
all required to effect relief from symptoms and a cure of the pathologic 
states primarily set up by the eye-strain. 

Taken at the onset of symptoms the results might have been pre- 
vented. These are the conditions which lie at the foundation of the 
modern requirement that no child shall be permitted to enter upon 



766 THE EYE AND NERVOUS SYSTEM 

school life until examination of the eyes has either excluded the 
existence of these congenital defects or secured their correction by 
glasses. 

The same chain of results may occur in adults without the existence 
of anomalies of refraction, through the neglect of suitable glasses after 
the onset of presbyopia; macular disease, hyperemia of the chorioid 
with occipital or frontal pain, often occur after forty years of age. 
The tougher sclerotic coat of adult life is not so liable to distention as 
in childhood and youth, but the reflex pain, hyperemia of the con- 
junctiva and impaired nutrition of the globe are equally imminent, 
and relief comes only after sufficient time has elapsed, under suitable 
hygienic conditions, to permit a subsidence of the pathologic condi- 
tions of the tissues. In many patients the use of glasses is not suffi- 
cient, but careful and persistent therapeutic measures are required, in 
addition to the rest from work. Time for recovery from the local, 
secondary results of eye-strain must therefore be allowed in many 
patients before it can be said positively that the eyes are not responsible 
for the headache. 

The subsidence of pain is not infrequently gradual, the paroxysms 
being less frequent, not so severe, and of shorter duration, until finally 
they fail to return, or only at long intervals, and then under special 
stress of circumstances. The etiologic relation of these ocular condi- 
tions to disease of the lining membranes of the bony sinuses of the 
skull which lie contiguous to the orbit is too frequently overlooked. 
That sinusitis is a frequent cause of certain forms of headache is well 
known, but that the eyes participate in the congestion due to blocking 
up of the exits from these sinuses into the nose has not met with the 
wide recognition its importance suggests. 

It will be observed in the foregoing paragraphs, therefore, that in 
the study and treatment of ocular neuroses many complex conditions 
are encountered which require a conservative application of knowledge, 
skill, and wide experience for their successful management. It is pos- 
sible that the ophthalmologist may be prone to exaggerate the im- 
portance of ocular affections as a factor in producing headache and 
general nervous symptoms, but this should not cause surprise when an 
analysis of one thousand private patients, applying consecutively for 
treatment, revealed the fact that approximately 50 per cent, of these 
complained of this symptom, and a considerable percentage of these 
headache patients came by the advice of their physicians and were un- 
conscious of any ocular defect. While his daily experience might there- 
fore lead him to overestimate the significance of eye-strain, it has taught 



LEUKOSES OCCASIONED BY EYE-STKAIJST 767 

him that there is great danger of its importance being overlooked or 
ignored by those laboring in other fields of practice. 

. There can be no donbt as to the fact that exaggerated importance 
has often been given to minor errors of refraction in patients whose eye 
symptoms, — e.g., hypersemia of the fundus, impaired function, head- 
ache, undue sensitiveness to light, etc., — were due to other causes than 
eye-strain. That these symptoms of eye disease are due to some general 
dyscrasia, — e.g., lithnemia, diabetes, and general blood-vessel disease, — 
is well known to all careful observers. 

That very small errors of refraction are frequently the cause of 
headache and other symptoms is true, but other sources of disturbances 
should be carefully excluded before ascribing the symptoms to eye- 
strain alone. Many persons with delicately poised nervous systems are 
nevertheless greatly benefited by the correction of very low degrees of 
astigmatism, particularly if the slight error of refraction is associated 
with ocular imbalance, and they are unable to abandon their glasses 
since any attempt to do so leads promptly to a return of their headache. 

In others, and they constitute a large group of persons who wander 
from one consulting room to another, the writer is convinced that the 
glasses afford the temporary relief which they claim to experience, by a 
species of suggestion, precisely as they would be relieved for a short 
time by the mummeries of a Christian scientist or the manipulations of 
an osteopath. Many women who have been despoiled of their ovaries 
belong to this group, and while they are often unable to go without 
their correcting glasses, never secure entire relief from their headaches, 
which they associate with any continued use of their eyes. 

The fact that many persons, even children, often abandon their 
glasses after wearing them for a few months, and that without return 
of their headache, is not to be accepted as proof that their symptoms 
were in the first place wrongly ascribed to eye-strain. Improved 
health, a long summer vacation, or change of environment, as for 
example, a better-lighted room or less pressing work, may enable them 
to bear the eye-strain with impunity, but the glasses had probably car- 
ried them over an important interval and prevented permanent injury. 

In treating headache demonstrably due to eye-strain, and for the 
present contention excluding from consideration all other possible 
causes and associated conditions, the writer will now pass to the discus- 
sion of the methods which experience has taught to be of greatest 
efficiency. 

In patients who suffer but little pain but complain only of blurring 
page after protracted effort at near-work, and in whom the ophthalmo- 



768 THE EYE AND NERVOUS SYSTEM 

scope shows a healthy eye ground, it is often sufficient to supply these 
with so-called manifest corrections; but in the class of persons under 
discussion in this chapter who, by reason of eye-strain are victims of 
headache and present the local inflammatory or irritative changes which 
have been described, it is necessary to use one of the mydriatic alkaloids 
to temporarily suspend the action of the ciliary muscle, in order to 
determine with accuracy the static refraction of the eye. 

The drugs usually selected for this purpose are the sulphates of 
atropia, hyoscyamine, and duboisine, and the hydrobromate of homa- 
tropine. Others are employed, but those named are more commonly 
used. These drugs possess not only the power of dilating the pupil and 
paralyzing the ciliary muscle, but are also local anaesthetics or analge- 
sics. Their value is twofold. In the first place they secure to the 
eyes a rest from accommodative efforts, and so relieve the eye-strain; 
at the same time through their soothing influence they aid materially in 
relieving the nervous irritability of the eye. It has been claimed in the 
foregoing pages that the immediate result of the accommodative strain 
constantly required to overcome some anomaly of refraction is to pro- 
duce hyperemia and turgescence of the ocular tunics, and that this, 
together with the exhaustion of the nerve-centres involved, is in great 
measure the cause of pain in the head and its associate reflex phenomena. 
The advantages therefore accruing to the patient from the use of the 
salts of mydriatic alkaloids are obvious. The pain in the eyes and 
head, the insomnia, the external redness and the turgescence of the 
uveal tract rapidly subside. These drugs should not, therefore, be 
regarded merely as cyclophlegics or paralyzers of the accommodation 
only, but as important and efficient therapeutic agents in the treatment 
of the ocular conditions associated with "headache. To the class of 
patients under study this contention is of signal importance, since for 
their relief approximate corrections of the errors of refraction are not 
sufficient. The exact nature and degree of the defect must be accurately 
known, and this cannot' be determined in a pair of congested, irritable 
eyes, with a fluffy chorioid, and exhibiting undue sensibility to light. 
Before any trustworthy data as to the static refraction can be secured, 
the eye must have time to recover from these conditions, and recovery is 
not only more rapid under the use of mydriatics than any other known 
means, but in most cases they are the sole means by which satisfactory 
results may be secured. This is particularly important where diseased 
changes at the macula are present. 

Many failures to demonstrate ocular responsibility for headache are 
due to neglect at this point. So-called manifest corrections of refrac- 



NEUROSES OCCASIONED BY EYE-STRAIN 769 

tion errors in this class of patients are valueless for diagnostic purpose^. 
All hurry-up methods are almost equally so. There is much variety 
in the opinion and practice of different ophthalmic surgeons as to the 
best methods of employing the cyclophlegics. Many prefer to use the 
stronger and more persistent members of the group ; for example, solu- 
tions of the sulphates of atropine or hyoscyamine, while others use 
almost habitually the hydrobromate of homatropine and consider it 
sufficient Homatropine possesses the sole advantage of being evanes- 
cent in the duration of its mydriatic and cvcloplegic properties. The 
writer demonstrated its efficiency, in 1881, for the determination of 
errors of refraction in healthy eyes, but in headache patients the eyes 
do not as a rule belong to this class. Eor these patients homatropine 
is not efficient unless, in strong solution frequently instilled, it is con- 
tinued day after day until the pathologic conditions of the fundus oculi 
have subsided, in which case it is better to adopt at the outset one of the 
stronger, more efficient, and enduring drugs. 

Then, too, after the measurements have been made, the correcting 
glasses ordered and the mydriatic omitted, another practical advantage 
is secured by the long-continued influence of the cvcloplegic. The 
glasses should be worn by the patient as soon as possible so that the 
power of accommodation shall be recovered under their use. 

An important factor in the disturbance of function in all cases of 
refraction error is the derangement of the binocular balance. That is 
to say, since the convergence and accommodation are under the same 
innervation impulse, the existence of any refraction error which in- 
creases or diminishes the amount of accommodation required to secure 
a clear image upon the fovea of each eye, must of necessity disturb the 
relation of the accommodation impulse to that required for convergence 
of the two eyes to the same point. 

Theoretically, therefore, every hypermetropic or myopic eye would 
have as an alternative a choice between a dim or imperfectly focussed 
image upon the fovea, or double images, for the reason that the eyes 
with a convergence impulse corresponding to the power of accommoda- 
tion required to be exerted for the clear image, would not be converged 
to the required point, but in hypermetropia to a too near point and in 
myopia to one too remote. The fusion sense has, however, taught most 
persons the trick, in hypermetropia, of accommodating within narrow 
limits without convergence, and in myopia to converge to the required 
point without accommodation, a fact pointed out by the investigations 
of Donders and designated by him as " the relative range and region of 
.accommodation and convergence." By this means the alternative of 
49 



770 THE EYE AND NERVOUS SYSTEM 

dim sight or double sight, in most individuals, is in large measure, or 
entirely, averted. This result of lifelong experience, however, becomes 
a disturbing factor when correcting glasses are attempted. As soon as 
the necessity for the accustomed undue strain upon the accommodation 
is removed by a convex glass, as in the correction of hypermetropia, it 
becomes necessary for satisfactory binocular vision that they should 
unlearn the habit of a lifetime and exert the same innervation impulse 
for both the convergence and accommodation, as in emmetropia. The 
great difficulty many persons have in learning to wear a pair of correct- 
ing glasses grows out of the fact that they cannot adjust themselves 
to this new order of things immediately. They do so, however, with 
much less difficulty if the glasses are worn during the days required for 
the cycloplegic to disappear. The gradually returning accommodation 
affords an opportunity for a readjustment of the new relations between 
convergence and accommodation. Having these considerations in view, 
the glasses for headache patients certainly should be prescribed at once 
and worn steadily. 

The careful study of the binocular balance is a most important 
phase of the treatment of eye-strain. It will be found, if appropriate 
means are employed, that a large percentage of patients applying for 
relief from eye-strain symptoms present some form of ocular imbalance. 
Esophoria and exophoria, or low degrees of vertical deviation are the 
rule. Fortunately, under the sufficiently prolonged use of a mydriatic, 
many of these deviations will be found to have disappeared when sought 
for through accurately adjusted glasses which correct the existing defect 
of refraction. There is nevertheless a large percentage of them which 
remain as absolute defects of balance between the two eyes. Those which 
disappear are related to the muscular strain and anomalous innervation 
impulses brought about by the attempt to overcome the defects of refrac- 
tion in the interests of clear vision. I have elsewhere designated them 
as relative heterophorias. When the strain is removed by glasses and 
clear vision secured without undue accommodation effort the tendency 
of the imbalance is to disappear, either spontaneously, or by suitable 
muscular exercise with prisms. 

The permanent defects of balance have, however, an anatomical 
basis, as already pointed out (see page 6) and require careful analysis 
and separate treatment. 

The following clinical history will serve to illustrate a somewhat 
numerous class of patients with defective vision who apply for relief 
from their painful eyes, headache, and a complex of symptoms which 
can be regarded only as ocular neuroses. 



NEUROSES OCCASIONED BY EYE-STRAIN 771 

Mr. K., aged 26, applied for treatment in December, 1899. He was 
wearing the following glasses ordered by Saemisch of Bonn, in 1894: 

O.D. -2.75° ax. 105° 
O.S. -1.75° ax. 90° 

with these V.=O.D. 6/60, O.S. 6/9. He suffered from constant fronto- 
occipital headache which frequently culminated in violent attacks of 
pain and general " nervousness" which drove him from business and to 
bed. He carried his head tilted to the right ; the eyes were red, unduly 
sensitive to light, and he suffered from blepharitis and recurring styes. 
The ophthalmoscope showed a fluffy, turgid fundus in both eyes, and 
in the right a distorted disc, and a large conus below, with concentric, 
crescentic markings indicating successive fresh encroachments upon 
the chorioid. The axis of the conus was at 120°. In the left eye the 
conus was at the temporal border of the nerve and beyond it ; approach- 
ing the fovea there were granular changes in the chorioid. The right 
eye was on a plane in front of the left, the right side of the face was 
larger than the left, the zygomatic arch being prominent, while on the 
left it was flat or slightly concave. The left eye was nearer the bridge 
of the nose than the right. The ophthalmometer indicated in the right 
eye a corneal astigmatism of 4.D., the principal meridians being at 30° 
and 120° respectively. In the left eye an astigmatism of 3.D. the 
meridians lying at 170° and 80°. After prolonged mydriasis and rest 
a careful measurement by objective and subjective methods resulted 
as follows : 

O.D. - .25 s C - 4.00 D. cyl. ax. 30° V. = ^4n 

V 1-I-2. 

O.S. - 1.25D. C + 2.50 cyl. ax. 80° V. = ^ 

Through these correcting glasses, carefully centred, the ocular imbalance 
was measured at 6 m . and revealed a left hyperphoria of 8° and an 
esophoria of 6°. These deviations would vary, the hyperphoria be- 
coming greater, while the esophoria diminished, or the reverse would 
occur, in the meantime the upper end of the vertical meridian of each 
cornea rotating outward (cyclophoria). The sketch on page 747, Fig. 
la, taken by his hatter, gives the skull outline. The septum, in the 
nose, was deflected toward the left side, and the left middle turbinate 
was firmly wedged between it and the bony wall of the nostril. The 
roof of the mouth was a deep cathedral arch. This case presents a 
somewhat exaggerated picture of the anatomical basis for many cases 
of anomaly of refraction associated with ocular imbalance. His father 



712 THE EYE AND NERVOUS SYSTEM 

and mother and a younger sister were subjects of hypermetropic astig- 
matism, and headache was a family trait. The eyes of this young man 
were evidently distending, the right having passed into myopic refrac- 
tion, and the left was myopic in the vertical meridian, the horizontal 
being still hypermetropic, — i.e., mixed astigmatism. The measurement 
and correction by glasses of the refraction error for each eye was not 
difficult, but the obvious difficulties presented in any effort to secure 
comfortable binocular vision illustrates forcibly the complex problem 
often presented in these cases of ocular imbalance. 

It is by no means always an easy task to discern the precise nature 
of the muscular defect, or which of the extra-ocular muscles is at fault, 
or whether the muscle or muscles at fault are so by virtue of impaired 
innervation, faulty development, or as the result of abnormal attach- 
ment to the globe. The analysis of the ocular movements, however, is 
often greatly facilitated by making the study at 6 m . while the accom- 
modation is still paralyzed. For this purpose it is important to employ 
some fixed form of apparatus in which the lenses required to correct 
the existing refraction error for each eye can be placed and carefully 
centred. The examination should be made with the eyes in the primary 
position, using a small point of light placed at six metres from the 
patient, preferably before a black background, from which all objects 
which may distract the attention are removed. 

Much skill, patience, and experience are required in the diagnosis 
and treatment of these cases of imbalance due to absolute muscular 
defects. In some instances, when there is much distortion of the walls 
of the orbit and of the peri-orbital region of the skull, it is probable 
that no amount of skill either by surgical interference or by other 
means can bring about comfortable binocular vision. 

One of these patients after many operations at the hands of several 
men of wide reputation came to the writer with the request that he 
should remove one of her eyes as the only means of promising any hope 
of relief from her distressing headache and nervous symptoms. The 
request was refused, but was subsequently granted by an eminent sur- 
geon in another city. It is probable that repeated tenotomies, advance- 
ment of tendons, etc., to which her eyes had been subjected had done 
harm, certainly had failed to afford relief. It is true, nevertheless, that 
there is a large number of patients who do not secure relief from accu- 
rate correcting glasses, whose continued trouble is unquestionably occa- 
sioned by a defective balance between the eyes which is susceptible of 
correction by surgical means. The power of convergence is insufficient 
or it is relatively too great for the power of abduction, or in the position 



NEUROSES OCCASIONED BY EYE-STKAIN 773 

of rest one eye is above the horizontal plane, or the other below it, and 
so binocular single vision is either intermittent or impossible, or is 
maintained by virtue of constant muscular tension which causes head- 
ache, etc. Exj^erience has shown that in the minor degrees of these 
faulty muscular conditions, the suitable use of prisms, combined with 
the glasses required for the correction of any existing error of refrac- 
tion, often give practically complete relief. In the higher degrees, 
however, surgical interference, designed to restore ocular balance, is 
theoretically a correct procedure and its value has been abundantly jus- 
tified by results. Where failure is met it may often be ascribed to mis- 
taken diagnosis, usually as to the nature of the fault the surgeon has 
sought to correct, or possibly to a faulty technique, or to some unavoid- 
able accident, — e.g., excessive hemorrhage into the capsule of Tenon, 
or to excessive inflammatory reaction during repair. In two cases seen 
in consultation, advancement operations had led to overcorrection of the 
defect through the contraction of the resulting cicatricial tissue. Mis- 
takes may occur in the selection of the wrong muscle for surgical pro- 
cedure. For example, tenotomy of an apparently too strong muscle 
may be performed when advancement of the weak one should have been 
the operation of selection, or, the. so-called secondary deviations may 
mislead the incautious operator. Then, too, it is not always easy to 
differentiate between deviations due to faulty attachments of a muscle 
tendon or a partial palsy of a muscle. 

It is not the intention in this chapter to present a treatise on either 
errors of refraction or the heterophorias, but to show the responsibility 
of these ocular abnormalities in causing ocular neuroses. In the pres- 
ence of these defects of vision the associated headaches should be 
regarded as possibly due to them until they have been corrected, and 
the resulting local pathologic states have been cured. 

Unfortunately, but little is actually known of the real mechanism 
of headache. Eor our present purpose, however, it is fortunate that 
the mechanism of eye-strain, if not of the resulting pain, is well under- 
stood. It is probable that no analogous conditions are presented else- 
where in the body. An illustration, however, of the possible results of 
eye-strain is afforded by writer's cramp. In the hypermetropic eye, 
for example, clear vision is maintained only by the constant contraction 
of the ciliary muscle. When astigmatism is also present, or if the 
defect of refraction is heteronymous, or of differing degrees between 
the eyes, or if the tormenting effect of ocular imbalance is added to the 
problem of maintaining clear vision, the difficulties of the struggle are 
greatly enhanced and the resulting exhaustion or derangement of the 



774 THE EYE AND KEKVOUS SYSTEM 

nerve-centres is more rapid and enduring. ~No mnscle or group of 
muscles in the body could endure with impunity the exhausting fatigue 
to which the ciliary muscles and their associated group of extra-ocular 
muscles are subjected, by the above described conditions, for the hours 
ordinarily required for the purpose of vision. To this muscular fatigue 
must be added the exhaustion of the nerve-centres involved in the 
innervation of the ocular muscular apparatus, the resulting congestion 
of the intra-ocular tunics and the consequent pressure upon the terminal 
distribution of the ciliary nerves, and possibly some subtle derange- 
ment of their nutrition occasioned by the impaired circulation produced 
by the increased intra-ocular tension. It is plain that in neurasthenics 
or feeble individuals, or in industrious people who use their eyes per- 
sistently under the added strain of near-work, the exhaustion would be 
relatively more complete and enduring than in vigorous and relatively 
indolent individuals. While the latter might endure with comparative 
impunity the imposed strain, the former groups of individuals would 
be forced into semi-invalidism. 



GENEEAL CONSIDEEATIONS : EPILEPSY; CHOEEA ; VEE- 
TIGO; GASTEIC DISTUEBANCES ; SPASMODIC MOVE- 
MENTS OF THE HEAD OTHEE THAN CHOEEIEOEM ; 
GENEEAL ASTHENOPIA. 

Part II. 
By HOWARD F. HANSELL, A.M., M. D. 

GENERAL CONSIDERATIONS. 

The tendency of modern medicine is encouragingly strong in its 
insistence that the diagnosis and treatment of disease shall be reduced 
to a demonstrable basis. Dogmatism and empiricism have been pushed 
farther and farther into the background of recent thought and litera- 
ture by the revelations of the microscope, the exactness of physiologic 
research, and by the accuracy of trained work and observation in the 
laboratory. Disease is not general to the system, but special to some 
part of it. It has a local, if not a discoverable, habitation in the human 
economy, and a cause which it is the mission of science to discover and 
remove. The more general recognition of this fact is bearing fruit in 
the minds and labors of medical men. Each year fewer diseases are 
classified by their symptoms, and more are referred to the category 
indicated by their etiology. As the causes of disease are laid bare, 
prophylaxis and treatment are simplified and become more efficacious. 
Surgery has broadened to the inclusion of affections that were con- 
sidered, not many years ago, to belong strictly to medicine, while the 
specialists are invading the old fields of both medicine and surgery. 
They are rightly accorded a more and more extended domain as their 
importance grows in recognition. This is particularly true of ophthal- 
mology. The well-directed and resourceful labors of earlier workers in 
this specialty, notably Thomson, Weir Mitchell, Dyer, Xorris, Harlan, 
and younger men, trained in their clinics and influenced by their 
teachings, have forced the acknowledgment of ophthalmology as an 
indispensable adjunct to the practice of medicine and surgery. Its 
services to the medical sciences is especially great in the diagnosis and 
treatment of the so-called functional diseases of the nervous system, 
and organic affections of the brain and abdominal organs. This 
specialty is also a brilliant contributor to the diagnosis of obscure 



776 THE EYE AND XEKVOUS SYSTEM 

organic lesions of the nervous system, circulatory apparatus and di- 
gestive organs. It reveals by the ophthalmoscope pathologic changes in 
the ocular tissues themselves, and in many obscure conditions discovers 
the active cause of a disease in an error of refraction or anomaly of the 
ocular muscles. Any theory of the origin of disordered function which 
does not embrace a consideration of the ocular apparatus is unscientific 
and open to criticism. There are incessant demands on the peripheral 
organs of vision which are in direct connection with the central nervous 
system, and the many ways in which this connection is provocative of 
disordered function is a fact of deep significance in the causation of 
disease. The cause or association is, moreover, continually active during 
the life of the individual. 

The etiological connection between eye-strain and reflex nervous 
disorders is well established and rests upon the foundation of extensive 
clinical experience. In no other branch of medicine is knowledge so 
demonstrably true. Given an optical defect, with a responsive or im- 
pressionable nervous system, reflex phenomena (the intensity and kind 
of which depend upon the character of the optical defect and the pecu- 
liarities of the individual) will be the invariable consequence. The 
degree of impressionability of the nervous system is of vital importance 
in the estimation of the relation of ocular cause to systemic effect. Not 
every error of refraction or anomaly of muscular balance, however 
severe, will give rise to reflex symptoms. The same degree of defect in 
a person of sensitive and nervous disposition that will originate dis- 
turbances in the functions of the body of no small import, will, in a 
phlegmatic, dull, unresponsive individual, give rise to no reflexes what- 
ever. It must not be forgotten, however, in determining the nature 
and severity of symptoms, that the power of observation and expression 
differ greatly in different persons, and one should not be misled by the 
failure of a patient to adequately express the feelings which demand 
the physician's attention. It may be necessary for the medical attendant 
to elaborate the facts without much reference to the ill-defined and 
misleading statements of the patient. 

The exhaustion or irritability of the nervous system consequent upon 
eye-strain is indisputable and manifold in its manifestations. In 
order thoroughly to comprehend ocular participation in the etiology of 
the disturbances of the functions of the body indicative of exhausted or 
perverted nerve action, the visual apparatus should be considered, (1) 
in relation to the function of accommodation in hyperopia, myopia, and 
astigmatism; (2) in the gradual failure of accommodation in advancing 
age; (3) in the associated action of the accommodative and extrinsic 



LEUKOSES OCCASIONED BY EYE-STRAIN 777 

ocular muscles; and in (4) the connection of the peripheral organs 
of vision with the central and sympathetic nervous systems. 

1. Emmetropia and equilibrium of the ocular muscles present ideal 
conditions. The emmetrope receives on his retina clear, denned images 
of distant objects (6 m. or more), with minimum effort of accommoda- 
tion and contraction of the extraocular muscles (equilibrium and 
binocular fixation). For objects nearer than infinity and to within 33 
cm. (the reading distance) of the eyes, the refracting power of each eye 
is increased by the uniform, gradual or sudden contraction of the ciliary 
muscles and the simultaneous contraction of the muscles of convergence, 
the equivalent relaxation of the muscles of divergence and the mainten- 
ance of the same visual horizontal plane for both eyes by equal tension 
of the muscles of supraduction and infraduction. 

That equilibrium and physiologic relation between the action of 
intraocular and extraocular muscles shall be originated and sustained, 
nerve stimulation and muscular response must be normal. Such a com- 
bination implies a healthy condition of the centres of vision, an unin- 
terrupted and unimpaired function of the efferent and afferent nerves 
and that their peripheral distribution is normal. The separate muscles 
of both eyes do not co-ordinate perfectly unless they have normal rela- 
tions with each other and with the eyeballs. The development of ocular 
reflex neuroses in emmetropia and extraocular muscle balance must be 
referred to an extraocular cause, such as the weakened or deficient action 
of the central nervous system, or of the circulatory apparatus, as in con- 
valescence from exhausting disease or confinement, affections of the 
organs of the chest or abdominal cavity, prolonged near use of the 
eyes among unfavorable surroundings, the gouty or rheumatic diathesis, 
venereal excess, overindulgence in stimulants or narcotics, or other 
source by which the visual apparatus may be deranged. The remedy is 
not far to seek. The restoration to health of the diseased organs, the 
avoidance of all excesses, and the use of the eyes under favorable con- 
ditions and for a reasonable length of time, will be followed by a gradual 
return to normal condition. 

In the presence of an error of refraction, the physiologic relations 
between the peripheral organ of vision, including its extrinsic muscles 
and the cerebral centres, are disturbed. For example, in hyperopia 
abnormal contraction of the ciliary muscle is required for clear vision. 
Thus the muscles of convergence receive an excess of stimulation, those 
of divergence a stimulation so modified that they may yield to the 
demand of convergence, and those of elevation and depression a degree 
of innervation corresponding to their anatomical relations with the 



778 THE EYE AKD NERVOUS SYSTEM 

muscles of convergence and divergence. The primary cause — hyperopia 
— thus initiates a series of morbid phenomena, abnormal muscular action 
and centrifugal impulse that eventuates in a deranged ocular function 
which, if not corrected, involves the general health and nervous organiza- 
tion, exhibited by definite, pronounced, vague, or bizarre reflexes. The 
eye-strain in myopia, is, conversely, due to the unassociated action of 
convergence and accommodation, the tendency to monocular vision and 
the intense effort to converge and maintain binocular singular vision 
for all distances. The eye-strain in astigmatism is that of hyperopia or 
myopia in conjunction with the unequal contraction, hence innervation 
of the ciliary muscle in the constant effort to obtain clearly defined 
retinal images. (See Chapter II.) 

The ill effects of the use of the eyes in all forms of ametropia are 
referable to the eyes as well as to the muscles which control their move- 
ments. In most cases an error of refraction is the primary cause, and 
muscular anomalies are secondary and dependent affections. In a small 
minority of cases, however, as in anatomical peculiarities of muscular 
insertion, malformations of the bones of the orbit or skull, or in muscu- 
lar weakness or paralysis, eye-strain may exist as the result primarily 
of muscular incoordination. These conditions, irrespective of the re- 
fraction of the eyes, react upon the central nervous system and occasion 
manifestations of disordered function exactly as though the muscular 
anomaly were secondary to ametropia. 

2. The symptoms of uncorrected presbyopia, or the gradual failure 
of accommodation in advancing years, are indistinct near vision, reces- 
sion of the reading distance, the need of stronger light and larger type, 
headache, and other forms of asthenopia. To these increasing disabili- 
ties may be added, in exceptional cases, any or all forms of ocular re- 
flexes common to ametropia and muscular imbalance. The direct and 
indirect symptoms are referable to the strain of an accommodative 
power weakened by lowered contractility of the ciliary muscle as a 
part of the general muscular loss of tone coincident with age, and to a 
gradual stiffening and loss of elasticity of the crystalline lens. Such an 
eye is no longer able to adapt itself quickly, if at all, to the demands of 
vision at close range, and the nerve stimulus to the ciliary muscle, prob- 
ably not lessened by age, meets with insufficient response. Hence, vision 
is blurred and cannot be cleared except by the employment of convex 
lenses. 

3. There is an interdependence of the functions of the intraocular 
and extraocular muscles. Every accomplished act of accommodation, 
and every rotation of the eyes in any direction, is consummated by the 



LEUKOSES -OCCASIOXED BY EYE-STRAIX 779 

co-ordinated contraction or relaxation of twelve extraocular muscles. 
Balance or equilibrium presupposes normal action of each of the muscles 
individually and all of them in combination, and equivalent and asso- 
ciated response of the ciliary muscles and irides. An abnormal demand 
on the accommodation, too great or too small, is responsible for hetero- 
phoria or heterotopia, both lateral and vertical. The tendency of the 
eyes to rotate inwardly (insufficiency of the externi), or convergence of 
the visual axes, is associated with excessive accommodative effort, while 
the tendency to outward rotation (insufficiency of the interni) in- 
volves little or no accommodative effort. Both inward and outward 
tendencies to deviation are combined with vertical insufficiencies in 
every instance, as all muscles governed by the same nerve respond to 
the stimulation, and individual muscles, or sets of muscles, must act 
jointly with all the extraocular muscles in contraction and relaxation. 
This close relationship of the ciliary and the extraocular muscles, and 
of the extraocular muscles to the nerves that govern them, is invariably 
deranged by errors of refraction. Ametropia, therefore, is responsible 
for accommodative strain and muscular asthenopia, the effects of which 
are by no means limited to the eyes, or to the head, but are reflected to 
the various organs of the body in such way as to interfere with their 
nutrition and function. The use of the eyes under these conditions of 
faulty focussing, or faulty rotation, in neurotic individuals, gives rise 
to local discomfort and reflex phenomena of various kinds. 

4. We have seen how errors of refraction and muscular anomalies 
may be the cause of asthenopia and reflex neuroses. Morbid states of 
the central and sympathetic nervous systems, on the other hand, are so 
well known that it is only necessary to allude to them as one of the 
factors in the development of ocular symptoms. Intracranial affections, 
for example, by pressure upon a nerve supplying an eye muscle, or 
involvement of the optic nerve directly or indirectly, spinal disease, 
affections of the circulatory apparatus or of the abdominal organs, will 
impair or destroy binocular vision or sight itself. The so-called con- 
stitutional dyscrasias, syphilis, tuberculosis, rheumatism, scrofula, dia- 
betes, anemia, etc., directly and markedly affect the organs of vision by 
inflammatory outbreaks and indirectly by vitiating the tone and health 
of the component tissues of the eye. The demands of modern business 
and active competition in all lines of work, the energy and unremitting 
industry essential to success, — in a word, the struggle for existence, — 
make too many and great and uninterrupted calls upon the vital forces of 
the individual. A little more time for relaxation, a little more freedom 
from care and responsibility in the great, and apparently necessary. 



780 THE EYE AND NEKVOUS- SYSTEM 

fight for individual supremacy in the daily affairs of business and pro- 
fessional life, would give much-abused nature the opportunity to assert 
her rehabilitating influences and thus enable the individual to preserve, 
unimpaired, his faculties and powers of endurance almost to the end 
of extreme old age. It is a noteworthy fact that in our strenuous Ameri- 
can life the eyes suffer first and most in the general break-down of the 
system. 

Low defects of refraction and comparatively insignificant muscular 
insufficiencies which, under healthier and more sanitary conditions 
would have remained in harmless abeyance, are provocative of ocular 
distress, headache, and other symptoms of a serious and more widely 
disseminated nature. 

An editorial writer in American Medicine, February 20, 1904, 
states the same thought in different language : " One Overlooked Factor 
in the Increase of Insanity. The new element in modern civilization 
which is not recognized and which is known to be a profound source 
of many derangements is school life and reading. The compulsory or 
chosen occupation of reading and near use of the eyes in a thousand occu- 
pations has been the dominant element in the life of the city dweller. 
Printing, books, schools, libraries, cheap books, newspapers, and handi- 
craft occupations have appeared and compel a physiologic or unphysio- 
logic activity which, whether harmful or not, is a new function, and 
one to which in evolution neither the mind nor the body has been 
habited, and especially to which the highest, most delicate and most 
used sense organ has never been bred. Moreover, this sense organ is 
demonstrably imperfect in a large proportion of all persons, and the 
action of .the malformed eye, as has been demonstrated by science, and 
by thousands of clinical cases, begets disturbances of eye, of cerebral 
action, of feelings, and of nutrition. Malfunction is the beginning of 
all disease, and of many it is the middle and end. Suicide, it has been 
found, is exactly in proportion to the number of hours of school life 
and study demanded in the country concerned. To this suggestive fact 
is now added the demonstration that insanity is almost precisely in the 
same proportion. When, lastly, it is observed that the use of the eye 
demanded by the civilization of the last century or two is one that its 
evolution has never provided for, either in structure or in physiology, 
one gets a glimpse of morbid mental action in the making. When it 
is found that the insane and criminal have an astonishingly high pro- 
portion of high optical defects, the suggestion grows to conviction that 
the morbid action of the eye and its resultant cerebral functions are 
considerable causes of the increase of insanity. 7 ' 



NEUROSES OCCASIONED BY EYE-STRAIN T81 

But the eyes are not peculiar as disturbing agents of the human 
body. Physical weakness in one part of the body interferes with the 
functions of the entire organization. Moreover, asthenopia, whether 
refractive or muscular, reacts upon the weakened body, and aggravates 
the symptoms of nervous exhaustion by still further reducing assimila- 
tion of food and constructive metamorphosis. In this sense, eye-strain 
is a cause as well as an effect. In health, on the other hand, ocular 
defects of considerable degree are suppressed, have no deleterious effects 
upon the nervous system, and are recognized only by a lowering of the 
visual acuity from failure of accommodation or from appreciable loss 
of muscular co-ordination. 

Eye-strain, productive of local symptoms and reflex neuroses, is thus 
accommodative and muscular, the former referring to overaction, and 
the latter to imbalance, or unequal tension of the muscles which govern 
the eyes in their rotations in the orbital cavities. Manifestly, one-eyed 
individuals are excluded from the second class. This fact applies to 
those who literally have but one eye, but also to persons who do not, 
under any circumstances, have binocular single vision. Muscular as- 
thenopia is also absent in paralytic or functional squint. This is ob- 
viously true, as the essential factor in the causation of ocular pain or 
distress is the maintenance of binocular fixation under the stress of 
muscular imbalance of a degree not incompatible with the simultaneous 
use of both eyes. The indirect cause of asthenopia is the tendency to 
turn the visual axes away from parallelism ; the direct cause, the tension 
of the opposing set of muscles, whose function it is to resist the ten- 
dency. For instance, esophoria, the tendency to cross the visual axes, 
creates an abnormal effort on the part of the abducting muscles in order 
to preserve binocular single vision, and it is this extra innervation, and 
extra muscular response, which is the source of the distress. And this 
is true whether the muscles of convergence are relatively strong or those 
of divergence relatively weak. 

As already stated, errors of refraction and muscular anomalies are 
the ocular causes of accommodative asthenopia ; of the former, hyper- 
opia and hyperopic astigmatism are of the most frequent occurrence ; 
of the latter, hyperphoria is most common. Among the general or con- 
stitutional causes may be mentioned neurasthenia, heredity, nervous 
affections, debility of any nature, sexual excess, gout, rheumatism, or 
any condition of the system in which the power of endurance is reduced 
below the healthy standard. Frequent contributory causes are constant 
application of the eyes in close work, prolonged reading of fine type, 
too much or too little light, fine sewing and embroidery, and reading in 



782 THE EYE AND NERVOUS SYSTEM 

moving vehicles, or with the head in a position either on a line with or 
below the level of the trunk. 

Inasmuch as a pure horizontal or vertical tendency of the visual axes 
may not exist [excepting in rare abnormal tensions and relaxations 
originating in anatomical abnormalities, as a separate and distinct con- 
dition, uncomplicated with an upward (in predominating lateral ten- 
dency) or a horizontal (in vertical) tendency], we are forced to deal 
with an obliquely directed tendency to deviation of the visual lines. A 
consideration of the heterophorias as being the dominant causes of 
asthenopia and reflex neuroses, necessarily involves a study of the 
relative strengths of adduction, abduction, and sursumduction for ob- 
jects at all distances. If in emmetropia adduction equals, approxi- 
mately, 24° and abduction 2°, esophoria would be due to insufficiency of 
the muscles of divergence. If right (supraduction) =5° (left infraduc- 
tion) and left supraduction = 3° (right infraduction) the elevators of 
the right eye or the depressors of the left eye would be at fault. If 
right supraduction (left infraduction) = 3° and left supraduction 
(right infraduction) =1° the fault would lie with the elevators of the 
left eye, or depressors of the right. For measuring the limits of rota- 
tion of the cornea in the horizontal and vertical meridians, the clino- 
scope of Stevens is valuable. By its reading, in degrees of the circle, the 
excursions of the eye may be readily compared. In addition to the alter- 
nate vertical tendency already mentioned, cases are met with in which the 
vertical inclinations are present and of the same direction in both eyes. 
Anaphoria is the name given to designate upward, and kataphoria 
downward, abnormal tensions of both eyes. Such conditions are the 
result of pathologic states of the muscles or nerves supplying them, 
or of some obscure unknown disease. They are not satisfactorily ex- 
plained by errors of refraction or by the muscular contractions induced 
by the effort to see clearly. It is of vital importance to know that such 
conditions exist, and that they may cause eye-strain and reflex 
anomalies. 

EPILEPSY. 

There is no uniformity among authors in their definition of epilepsy. 
It is hardly possible to define it exactly. Epilepsy is a symptom ex- 
pressive of an organic or functional derangement of the nervous system. 
It is not a disease per se, etiologically or pathologically, but a symptom 
complex. One of the tersest, most accurate, and comprehensive de- 
scriptions of the commonly accepted meanings of the word is given nega- 
tively in Gower's " Diseases of the Nervous System," published in 1888. 
:c The term epilepsy," he writes (page 1078), " is applied to a disease 



NEUROSES OCCASIONED BY EYE-STRAIN 783 

in which there are convulsions of a certain type, or sudden loss or im- 
pairment of consciousness, but in which the convulsions are not directly 
due to active organic brain disease, to reflex irritation, or to abnormal 
blood states, and in which the loss of consciousness is not due to primary 
failure of the heart's action." Subsequent writers have not improved 
this definition. H. C. Wood defines epilepsy 1 as " A disease of un- 
known pathology in which, at irregular intervals and without obvious 
existing causes, an abnormal disturbance of nerve force occurs, in most 
cases accompanied with loss of consciousness, and very frequently with 
convulsive disturbance." Landon Carter Gray 2 admits that " much 
confusion has been injected into the subject by the failure to recognize 
that epilepsy is only a symptom, just as is fever or cough, and that in 
every case, the question of diagnosis is resolved into the further one of 
what the epilepsy is a symptom of." Other authors might be quoted 
without adding useful evidence to the universally admitted opinion that 
the so-called disease, epilepsy, is the accompaniment of not one but many 
affections, in some of which organic changes in the neryous system are 
found after death, while in others failure to recognize that any of the 
tissue is diseased is the only result of microscopic examination. 

Epilepsy, as a symptom of disease, has no pathology. Authorities 
are conflicting and inconsistent as to this question. It is, for instance, 
classified as a functional neurosis, and yet pages are devoted to the dis- 
cussion of its pathology. Local and general convulsions are due, in 
many cases, to organic changes in the brain or spinal cord, and are un- 
scientifically classified as functional affections, when they should be 
designated, for example, as " epileptic convulsions in hemiplegia." 
Exactly identical convulsions may appear as reflex symptoms of some 
peripheral lesion. A wider recognition of these facts would lead to a 
more tenable classification of epilepsy than that usually given, and 
would tend eventually to eliminate such objectionable adjectives as 
idiopathic. The subdivision of the subject would then be " Epilepsy 
from organic disease of the nervous system" and " Epilepsy from 
peripheral irritation," although the exciting cause might remain un- 
known in many cases. 

The testimony of too many trustworthy clinicians has been pub- 
lished in confirmation of the statement that epileptic convulsions may 
be due to affections of other organs than the brain and spinal cord, to 
permit the causative relation of distant lesions to the development of 
the affection to be ignored. It must be admitted, however, that some 

1 Pepper's System, Theory, and Practice of Medicine, 1893. 

2 Nervous Diseases, by American Authors, edited by F. X. Dercum, 1S95. 



784 THE EYE AKD NEKVOUS SYSTEM 

other factor, aside from the peripheral lesion, must be active. In 
considering the nature of the factor, we must assume that some state or 
condition of the nervous system makes it peculiarly receptive, impres- 
sionable, and responsive. And the question arises, What are the pre- 
disposing and underlying causes of epilepsy? We confess our ignor- 
ance here, as we are forced to confess it in many other affections, and 
expose our lack of exact knowledge by the use and abuse of such 
names as heredity, susceptibility, idiosyncrasy. We cannot explain by 
the results of pathologic examination or post-mortem studies why an 
old cicatrix should cause epileptic attacks in one individual and not 
another, or why intestinal worms will cause corneal ulcer in one child 
and not in all children, without having recourse to the intangible but 
none the less cogent influence of personal susceptibility, an indefinable 
quality, inherent and peculiar to the individual. 

Etiology. — In some respects the theories of the diagnosis and treat- 
ment of functional neurosis need reconstruction. It can certainly not 
be claimed by any enlightened medical man, however earnest and 
enthusiastic he may be, that all or even a large proportion of " idio- 
pathic" cases are the reflex result of perverted function of some distant 
organ. On the other hand, in view of the authoritative statements and 
experience of the general practitioner, it cannot be doubted that at 
least a minority of such affections are reflex in origin. H. C. Wood 3 
divides epilepsy into organic, toxsemic, Jacksonian, idiopathic, and 
reflex, and gives as causes, wounds of the head or other portion of the 
body, astigmatism or other imperfection of the eyes, diseases and mal- 
formations of the nasal cavity, carious teeth, retained milk teeth, aural 
diseases, adherent prepuce or other irritation of the genital organs, and 
intestinal worms." W. B. Pritchard 4 gives as causes of reflex epilepsy, 
the existence of which, he says, is somewhat widely questioned, phimo- 
sis, adherent prepuce or clitoris, stenosis of uterine os, nerve cicatrices, 
or surgical disease of the limbs or joints implicating nerve trunks, 
carious teeth, eye-strain (esophoria, exophoria), obstructive diseases of 
the nasal passages, aural disease, etc., etc. Herter and Smith, 5 in 
considering the etiology of epilepsy, mention as one of the most im- 
portant causes " putrefactive changes in the intestinal contents, toxic 
substances produced in the intestines" (reflex epilepsy). Stoewer 6 says 

3 Pepper's Text-Book. 

4 Sajous's Annual, vol. iii., 1899. 

5 New York Medical Journal, August and September, 1892. 

6 Augenkrankheiten als Ursache der Epilepsie, Klin. Monatsb. f . Augenh., 3G, 
1896. 



LEUKOSES OCCASIONED BY EYE-STRAIN 785 

" most cases are laid to the cerebrum, while for other cases, certain irri- 
tations of peripheral nerves are necessary for outbreaks of an attack/' 
and " many of the last reflex class are cured by the removal of painful 
scars, irritation from nose and ear." P. F. Barbour 7 reports the case of 
a male, married, who had epileptic seizures since 1892. He found a 
marked increase of indican in the urine and dilatation of the stomach. 
Hence he inferred that the trouble arose from intestinal indigestion and 
constipation. Treatment for these conditions relieved the epilepsy. 

These quotations and abstracts, and more might readily be added, 
are sufficient to prove that epileptic attacks may be reflex in origin, and 
that removal of the source of irritation will re-establish normal function 
of the nervous system. It is, therefore, logical to assume, and the 
assumption has been abundantly verified, that reflex epilepsy may be 
the result of eye-strain, accommodative and muscular. The relation 
of the function of the ciliary muscles to the extraocular muscles, has 
already been alluded to. Most cases of accommodative asthenopia are 
also cases of muscular asthenopia. Therefore, both causes may be 
active in the same individual, and should be taken into account as 
factors. The relative importance, in the etiology of nervous affections, 
of refraction and muscular anomalies, is difficult to determine. Some 
authors ascribe the whole responsibility to refractive errors, others to 
want of equilibrium of the extraocular muscles, and not a few writers 
utterly ignore the eye and its muscles. Strohmayer, 8 for instance, dips 
deeply into the consideration of etiology and treatment by medical and 
hygienic measures. The omission in a systematic monograph of such 
recent date is surprising, and the only inference, of course, is that 
the writer does not believe in eye-strain as a cause of epilepsy. Landon 
Carter Gray 9 makes a distinction between the two sources of eye- 
strain. " The removal of insufficiencies of the ocular muscles is, in 
my opinion, quite useless, but marked errors of refraction should be 
remedied." The reasonableness of this decision, half-hearted though it 
is, may well be questioned. The dual cause is so intimately and so 
inseparably bound together, that a separation or elimination of either 
is not only unscientific but inconsistent with intelligent criticism. 

M. Allen Starr 10 believes that epilepsy is an organic disease. He 
bases his opinion on the study of 4000 cases and his researches are 

7 Medical Times, February, 1895. 

8 Die Epilepsie im Kindesalter, 1892. 

9 Nervous Diseases, by American Authors. 

10 New York Medical Journal and Philadelphia Medical Journal, January 30, 
1904. 

50 



786 THE EYE AND NEKVOUS SYSTEM 

certainly worthy of careful attention. One hundred and ninety-two of 
the 4000 cases exhibited positive signs of non-development of the brain, 
68 per cent., signs of epilepsy before twenty years of age, the period of 
brain development. The most frequent cause was heredity, and the 
next in frequency, trauma of the head. That the latter was a more 
potent cause than wounds of other parts of the body, was well shown by 
the statistics of the Eranco-Prussian war. These were conclusive. Out 
of 8889 soldiers wounded in the head, 46 became epileptic; whereas, 
out of 77,463 persons wounded elsewhere, only 17 became epileptic. 
Fright was undoubtedly a cause, as 119 of the patients tabulated had the 
first fit after a fright. 

C. L. Dana, in discussing Starr's paper, said, "At least ten years 
ago he had spoken of epilepsy as a progressive degenerative disease of 
the brain, but, as we did not yet know always what this degeneration 
was or where it was, and could not give a morphological description of 
the diseased cells, it was probably wise still to describe epilepsy as a 
disease without definite organic basis. He also doubted if the term 
disease was the proper one to use in connection with epilepsy. There 
was certainly an underlying organic degeneration, but it seemed to him 
that in many cases it was rather a morbid condition than a pathologic 
process, rather a teratological defect than an actual disease. So far 
as we know, it was probable that the most important factor underlying 
epilepsy was the tendency of large groups of nerve-cells to undergo a 
rather progressive degenerative process. With regard to the incurability 
of epilepsy, he expressed the opinion that the stand taken by Starr was 
not only incorrect but discouraging. He absolutely knew of cases in 
which epilepsy had been cured. He knew personally of a young man of 
seventeen, who had nocturnal epilepsy for one or two years, the attacks 
coming as often as two or three times a month. He had seen the attacks 
himself and they were characteristic. Under the bromide treatment 
this man recovered and was now forty-five years of age, and had never 
had an attack since completing the treatment. There was now none 
of the interconvulsive symptoms of epilepsy, and this disease should be 
looked upon as cured, just as much as pneumonia should be considered 
cured. The very theory of the disease, assuming it to be organic, would 
make a cure possible. We know perfectly well that there are certain 
degenerative processes that we may arrest; for example, tabes and de- 
generative processes affecting the kidney or liver, for degenerative pro- 
cesses were not always progressive." W. H. Thomson, in the discussion, 
said that he considered functional nervous diseases as true derange- 
ments of the source of nerve function, independently altogether of the 



NEUROSES OCCASIONED BY EYE-STKAIN 7S7 

structure of the brain, — i.e., the bloocl. Functional disease might be so 
serious as to terminate life, and yet no nerve cell or fibre could be 
demonstrated to be diseased, the disorder, perhaps, having been alto- 
gether due to a toxin in the blood. He could not believe that a disease 
so strikingly characterized by intermittency could consist essentially 
in a structural lesion, nutritive or otherwise. He . fully agreed with 
Dr. Starr that epilepsy was not curable by the surgeon, and stated on 
the contrary that a surgical operation on the brain was apt to be fol- 
lowed by epilepsy. A polypus of the nose sometimes gave rise to an 
intermittent asthma. He had had a number of cases in which the 
removal of peripheral irritation had been followed by the permanent 
disappearance of epilepsy. With regard to the pathology of epilepsy, 
we were often misled in regarding epilepsy as due to a discharge in the 
cerebral cortex. His definition of ej)ilepsy was a " sudden derange- 
ment of the normal inhibition of cortical centres upon one another, 
dependent in the first instance upon an abnormal afferent impression." 
R. H. MacAllister 11 analyzed 250 cases of epilepsy treated in C. L. 
Dana's clinic, and private practice, and does not mention ocular affec- 
tion among the etiological factors. 

Published cases of the cure of epilepsy by the removal of peripheral 
irritations are not uncommon, and include many varieties of lesions. 
Pecho 12 saw patients recover after enucleation of an atrophied ball, 
following an injury in early life. Galeowski 13 records the history of 
a man, aged forty, whose right eye was injured six years before, and 
for which abscission of the anterior part of the eye had been performed. 
The stump of the right eye was still very sensitive. For six months 
before he came under the care of Galezowski he had frequent epileptic 
seizures, and vision in the left eye had been failing. The ophthalmo- 
scope showed left neuroretinitis, venous congestion, perivascular infil- 
tration, and retinal hemorrhages. The stump of the right was sensitive 
to pressure. After enucleation of the stump, the epileptic attacks dis- 
appeared and vision in the left eye improved. Stoewer, reported the 
case of a married, healthy man thirty-two years old, who had been 
all his life myopic. In 1891 influenza had been followed by an in- 
tractable iritis and that in turn by epileptic seizures every few days, 
headache and eyeache. The eye was blind from closure of the pupil. 
He had tonic and clonic spasms, in which he became unconscious, and 
after recovery was extremely forgetful. He was treated by suggestion, 

11 New York Medical Journal. January 29, 1894. 

12 Rec. d'Ophthal., January, 1886. 

13 Rev. d'Ophthal., January, 1886. 



788 THE EYE AND NERVOUS SYSTEM 

electricity, and bromides, without benefit. On the contrary, the attacks 
became more frequent and violent. The patient became exhausted and 
emaciated. The attacks immediately ceased, and improvement in 
health marked, after enucleation of the left eye. Knapp 14 says, 
" There is a class of cases, however, in which the reflex origin seems 
fairly probable, — -.the cases of so-called epilepsy. A critical examina- 
tion of the cases reported under this head would eliminate many of 
them. A considerable number of them are undoubtedly hysteria, and 
in others the dependence of epilepsy upon the alleged source of irrita- 
tion is certainly doubtful. Nevertheless, the theory that a remote irrita- 
tion may give rise to convulsive seizures is supported by the well-known 
experiments of Brown-Sequard, who produced epilepsy in the guinea-pig 
by injury of the sciatic, and by a limited number of authentic cases in 
man. In most cases cited, the source of the epilepsy has been of trau- 
matic origin, and upon removal of the source of irritation, the epilepsy 
has ceased. These cases are, however, extremely rare." 

That heredity is an important predisposing factor is shown by 
Hare, 15 who found, after examination of many cases of epilepsy, that in 
25 per cent, a distinctly neurotic family history could be traced. 

The above extracts are sufficiently numerous and trustworthy to 
prove that the symptom complex known as epilepsy may be caused by 
peripheral lesions in persons who have neurotic tendencies. 

Science has been, as yet, unable to discover the post-mortem lesions, 
or conditions which explain the symptoms of epilepsy in the so-called 
functional forms. If it were possible to distinguish between func- 
tional and organic epilepsies, the whole subject would be elucidated. 
And yet, no description of epilepsy has been considered complete with- 
out a detailed account of the post-mortem findings. The pathology of 
epilepsy is that of the disease which causes the convulsions and is, in 
many cases, unknown. Gowers well says that " the changes in the 
nerve-centres are probably of that kind which is revealed only by 
altered function and eludes the most minute search. In the absence of 
any help from anatomy and histology, the pathology of idiopathic 
epilepsy is a matter of hypothesis." With the organic form of the 
affection we have nothing to do in this paper, and confine our observa- 
tions to functional neuroses as indications of peripheral irritation. 
In such affections there is primarily no pathology ; secondarily, as the 
result of the habit of convulsions, associated with unconsciousness, the 



"American Journal of the Medical Sciences, October, 1895. 
15 Quoted by Wood in Pepper's Text-Book. 



NEUROSES OCCASIONED BY EYE-STRAIN 789 

cortex may undergo changes which later develop into appreciable ana- 
tomical lesions. 

Although the vasomotor theory of the causation of the nerve ex- 
plosions has been given up as explanatory of epilepsy, it is still more 
applicable than any other theory to explain the condition of reflex 
epilepsy. The influence of the vasomotor nerves, originating or passing 
through the ganglia of the cervical sympathetic, is not thoroughly un- 
derstood. The effect of exsection of the superior ganglion upon the 
ocular tissues, however, is sufficiently pronounced to warrant the in- 
ference that the nerve impulse that passes out of the ganglion to the 
cerebral vessels, is modified by the condition of the ganglion, whether 
diseased or healthy. It may be here incidentally stated that in seeking 
to find a cure for idiopathic epilepsy surgeons have removed the ganglia 
in order to note the effect of its absence upon the calibre of the cerebral 
blood-vessels. The results of sympathectomy upon the eye are thus 
summarized by De Schweinitz : 16 " Myosis, narrowing of the palpebral 
aperture, projection of the nictitating membrane, diminished intra- 
ocular tension, certain ophthalmoscopic and microscopic lesions in the 
eye-ground, and possibly trophic disturbances. 1 ' 

The causative relations of derangements of the ocular apparatus to 
epilepsy have been the subject of no little controversy among neurolo- 
gists and ophthalmologists. Since the publication of Stevens's prize 
essay, and the investigation of Stevens's claims by the New York 
Neurological Society, the attention of the profession has been directed 
toward eye-strain as a cause of epilepsy, the relief of the convulsions 
by correction of optical errors, the restoration of balance of the extra- 
ocular muscles, and of the normal relationship between the intraocular 
and the extraocular muscles. The value of Stevens's labors has not 
been fully appreciated by the general practitioner, or even by the 
neurologist, and the ophthalmologist's efforts in this field are limited, 
except as employed in conjunction with his colleagues. The same idea 
is more forcibly expressed by R. H. Gibbons: 1T "I know a young man 
cured of epilepsy in a bad form by Ranney, and I know of four cases 
cured by Stevens. None of these cases has been reported and one of 
them has been cured for five years. Stevens and Ranney have been mis- 
quoted, or worse, — they have been misjudged by ophthalmologists in 
general and by neurologists in particular." The laity has already 
learned bv experience to apply to the oculist for relief of headache, but 



16 The Relation of the Cervical Sympathetic to the Eve, Ophthal. Sec. Amer. 
Med. Asso., 1903. 

"Trans. Penna. State Med. Soc, 1902. 



790 THE EYE AKD KERVOUS SYSTEM 

is not sufficiently advanced to appreciate that other neuroses may 
originate in eye-strain. The general practitioner must take the initia- 
tive, and, in the treatment of epilepsy, should endeavor to discover and 
probe every possible cause, including the ocular apparatus, and not 
wait until all the text-books on etiology have been exhausted before 
turning the eyes in the right direction. The writer has natural hesita- 
tion in expressing himself in a definite, practical manner as to the 
relation that eye-strain bears to the causation of epilepsy. Evidence 
culled from books and monographs is conflicting. It must be admitted 
that advocates of this theory of causation have not been successful in 
carrying conviction to the minds of men who have had most to do with 
epileptics. The theory has undoubted claims to careful and impartial 
consideration, and the work done along these lines is of sufficient weight 
to compel belief or refutation. The writer's own belief is, that a small 
proportion of cases of epilepsy, and of epileptiform convulsions, has 
been cured by eye treatment alone, and that the proportion equals, in 
percentage of cures, that of any other single surgical or mechanical 
means. He strongly advocates, moreover, a thorough and systematic 
ocular examination in all patients in whom the etiology of the convul- 
sions is unknown. In forming this opinion, he is guided, not by his own 
experience, which is most limited, but by the cases and histories re- 
ported in recent literature. Stevens 18 examined the ocular conditions 
in 140 cases of epilepsy. " The general result has been to reveal the 
existence, in these cases, of refraction anomalies in a considerably greater 
proportion than has been found by Cohen in his examination of the 
eyes of school children, or by other observers in similar investigations 
prosecuted in Germany, Russia, and America. In 100 consecutive 
cases, hyperopia was present in 59, myopia 23, emmetropia, or refrac- 
tion less than 1 D., 18. Twenty-nine cases were treated only by the 
removal of optical defects. Of these, 14 may be considered well, 2 
(still under observation) are believed to be permanently relieved; 3, 
still under treatment, have received such marked relief that it is be- 
lieved an entire discontinuance of the malady may be expected. One 
died, 7 received temporary relief, and 2 manifested no improvement. 
Some remarkable histories and striking photographs are appended to 
the histories of a few of the most pronounced cases. Ranney's sum- 
mary of 20 cases 19 is as follows: Four abandoned treatment. Of the 
22 remaining cases, 10, or 45 per cent, may be considered as well; 7 
being completely cured and 3 being practically cured ; amelioration of 

18 Functional Nervous Diseases, 1887. 

19 Reported in "Eye-Strain in Health and Disease," 1897. 



LEUKOSES OCCASIONED BY EYE-STRAIN 791 

the attacks has been afforded by eye treatment in 9 cases, or nearly 42 
per cent. ; no improvement has been observed in 3 cases, about 13 per 
cent. Ranney's collection of case histories is truly a remarkable one, 
and, viewed from an unprejudiced stand-point, convincing. As he says 
in the general resume, " I have brought forward here some very strong 
written testimony from physicians of repute, and the patients them- 
selves, to prove that the histories published by me are those of genuine 
epileptics, that the results were as I stated, and that benefit followed 
the relief of eye-strain after a failure of medicines or diet to control the 
seizures." J. E. Colburn 20 examined 43 cases of well-marked epilepsy, 
and from this number selected 14 which presented subjective and ob- 
jective symptoms of eye-strain. Those rejected, 29 in number, pre- 
sented other causes for the epilepsy or failed to afford evidence that the 
eyes, or their function, were abnormal. The patients who submitted to 
the eye treatment had refractive or muscular anomalies, or both, and 
were treated by glasses, prisms, or tenotomies. His results were as 
follows: 8 cases had no return of the convulsions or other indica- 
tions of a continuance of the disease for a period of from two to six 
years ; 6 were benefited in a general way and the attacks rendered less 
frequent ; one relapsed as soon as the glasses were withdrawn ; one 
received no benefit ; one, a case aggravated in every respect, could not 
be held under observation. The same author reports 21 a case of 
epilepsy cured by correcting an error of refraction, the inherited neu- 
rotic taint being especially prominent in the family of the patient. A man, 
aged twenty-four, complained of mental confusion, flushing of the face 
and vertigo in attempting to read, and remarked that the symptoms dis- 
appeared when the eyes were closed. At times he was dull and morose, 
and at others brilliant and exemplary. The diagnosis was that of " petit 
mal with impulses." The patient had been confined in an asylum at 
intervals for two years on account of epileptic seizures. Examination 
of the refraction showed hyperopia .50 D. of manifest, 2 D. of latent; 
esophoria 8 degrees. By restoration of the balance of the ocular 
muscles by prism exercise and correction of the total H., the man was 
relieved of his pitiable condition and was made strong and well. The 
patient's mother suffered from neurasthenia and " masked" epileptic 
convulsions, vertigo and unsteadiness of gait. She had H. 2 D., HAs. 1 
D., and a muscular error. After the optical defects were fully corrected 
she, also, became well. The patient's sister was emmetropic with 
neurotic tendencies. A brother had been an inmate of an asylum for 

20 Chicago Medical Record, 1894. 

21 Peoria Medical Monthly, February, 1888. 



792 THE EYE AKD NERVOUS SYSTEM 

some form of insanity. Colburn says, " The hereditary hyperopia 
error of refraction was responsible for all the symptoms of progressive 
cerebral disease, up to insanity." C. M. Capps records two cases. 22 
One was that of a girl of nine who consulted him one year before re- 
porting the result of the treatment. She had three to six epileptic 
convulsions every month since four years of age. Since entering 
school the seizures had become more frequent and violent. The seizures 
ceased while the accommodation was paralyzed with atropin, and re- 
mained absent during the year, while wearing correction for M. and 
MAs. The second case was a girl of eight who, after correction of II. 
and II As., had complete cessation of epileptic attacks. W. F. Connors 
describes the interesting case of a young man 23 who was cured by 
wearing correction for high H. Removal of the glasses was imme- 
diately followed by the prodromes of an epileptic convulsion. Reber 24 
narrates four cases that are worthy of brief description. Case 1, mar- 
ried woman, aged thirty, high hyperopic refraction combined with mus- 
cular imbalance, treated by spherocylinders and prisms ; result, " with- 
out any suggestion that her epilepsy might be benefited and with the 
withdrawal of all drugs, this woman was rescued from a future that 
was very gloomy at best. She had been having two or three seizures 
weekly, when she would fall to the floor unconscious and remain in a 
stupor for an hour or more. To be brought from that condition to the 
present one (having one fleeting fit a month, without loss of conscious- 
ness) is practically a cure." Case 2, unmarried woman, aged twenty, 
in apparently perfect health with the exception of nocturnal epilepsy, 
having had for a long time 12 to 14 seizures every week. The fre- 
quency of the attacks was reduced to one per week by wearing myopic 
cylinders. Later, prisms were added to her glasses. After wearing the 
combination for three months, she reported that she was virtually free 
from attacks, having had them only at great intervals and very lightly." 
Case 3, a strong, active youth of twenty. After reading several hours 
in the evening he would suffer on the next day several attacks of petit 
mal. Eor several years he had the attacks at irregular intervals, some- 
times one or two, sometimes several weeks apart. Treatment of mus- 
cular imbalance by tenotomies reduced the seizures to one or two slight 
attacks in over two years, so that, to all intents and purposes, he is a 
cured man. Case 4, a man of twenty-one, one of a family of eight 
children. " There are several neurotics in the family, and the general 



New York Medical Journal, September 16, 1899. 
Medical News, November 4, 1893. 
Pennsylvania Medical Journal, November, 1902. 



LEUKOSES OCCASIONED BY EYE-STRAIN 793 

ancestral history is studded with neurasthenia, hysteria, and chorea, 
but no epilepsy or alcoholism. At seventeen, he commenced to have 
epilepsy, having two seizures within 24 hours of each other every three 
or four weeks. Under partial prismatic correction of exophoria and 
withdrawal of drug treatment the attacks ceased for four months, when 
two very light attacks were induced by excessive reading several nights 
in succession. Instead of the 8 to 10 attacks he would- have had within 
the 19 months, taking all the time heavy doses of bromides, he had two 
very light seizures, almost imperceptible, and that without any drug 
treatment at all." E. G. Murphy writes 25 that in the past two years 
he had five epileptics under his care, four of whom had defective 
eyes. Two of these were completely relieved of all their epileptic 
symptoms by the correction of their optical defects. The remaining 
two wore their correction with relief of ocular symptoms, but none 
whatever from epileptic seizures. Ranney reports 26 results of eye treat- 
ment in 29 cases : 7 completely cured, 3 practically cured, 9 improved, 3 
not improved, 4 not counted. Gould 2T claims to have cured six cases, and 
in all " the cure was immediate, certain and complete and persisted for 
several years." He agrees with most ophthalmic surgeons that in ex- 
ceptional cases the ocular condition may be the pernicious factor that 
maintains the epileptic manifestations. The obligation is imperative 
that in every case of epilepsy the condition of the eyes should be care- 
fully considered. In the Spratling and Gould report of the eye ex- 
aminations and results of eye treatment in 78 selected cases of epilepsy, 
who were patients in the Craig Colony, 28 the same 'differences of opinion 
that characterizes similar reports are found. Marlow 29 corrected the 
refraction of a neurotic patient. The patient gained 20 pounds in 
weight as a result of wearing the glasses, and at the end of five years 
continued free from the intense nervousness and occasional epilepsy 
that had baffled many jDhysicians for some years. Coover details 15 
cases 30 of reflex irritation accompanying eye-strain. Among the promi- 
nent symptoms, all of which were relieved by correction of refractive 
error, were attacks of convulsions resembling epilepsy caused by con- 
tinued use of the eyes at the near point. Elmer 31 recited instances of 
patients afflicted with epileptic seizures, of mental conditions approach- 

25 Trans. Iowa State Med. Soc., vol. xvi., 1898. 

26 New York Medical Journal, January 2 and January 9, 1897. 

27 Journal of the American Medical Association, July 5, 1002. , 

28 American Medicine, April 9, 1904. 

29 Medical News, July 4, 1897. 

30 Medical News, October 26. 1895. 

31 Amer. Jour. Ophthal. and Otol., April, 1896. 



794 THE EYE AND KERVOUS SYSTEM 

ing insanity, of chorea, and of petit mal, who were entirely relieved 
by correcting glasses. 

Having thus presented some of the evidence published in advocacy 
of the treatment of the eyes as a means of cure of epilepsy, the other and 
negative side claims attention. Peterson 32 does not remember to have 
seen among several hundred epileptics a single case of genuine reflex epi- 
lepsy. Gowers 33 says, "It is extremely rare to meet with any evidence 
of a reflex cause in the system of the cerebrospinal nerves and in the 
few recorded cases there has been a manifest and strong predisposition." 
Starr believes that reflex neuroses of an epileptiform type are ex- 
ceedingly rare, and Dana says " the importance of reflex irritation has 
been much overestimated." Sachs does not even mention reflexes among 
the causes of epilepsy, except in a footnote, where he says the disease 
is rarely established by peripheral irritation. Concerning eye con- 
ditions, he says that they may cause single attacks, but they surely are 
not the cause of epilepsy. He asserts that Ranney does not cure cases, 
and makes the statement on the ground that Ranney has not observed 
them long enough. Both Dana and Sachs reiterate these opinions in 
their latest published articles. 34 Fere 35 as late as 1897, in his full 
chapter on epilepsy, says that " errors of refraction, necessitating as 
they do efforts of accommodation, may cause epilepsy," and, in the 
section devoted to treatment, " anomalies of refraction must be cor- 
rected." He does not include anomalies of the ocular muscles among 
the causes. Gowers, in his monograph, 36 is silent on the subject. Sink- 
ler and Pearce, 37 in their analysis of 315 cases, also pass ocular causes 
by without comment. H. A. Hare 38 quotes Stevens as to the relation 
of muscular anomalies and refraction errors to the production of epi- 
lepsy, and says that thus far Stevens's remarkable cases have adduced 
no evidence in rebuttal, but in the section on treatment, he does not 
refer to the ocular treatment, as one of the means of relieving epilepsy. 

Thus, it will be seen that the proof of the causal relations of affec- 
tions of the eye to epilepsy rests entirely with the ophthalmologists. 
The ablest among the neurologists barely refer to the eye as a cause, or 
omit it altogether. An unbiased opinion formed from a study of the 
literature on both sides of the question, and expressed tersely, would be 

32 New York Medical Journal, July 4, 1896. 

33 Mental Diseases. 

34 Medical News, July 30, 1904. 

35 Twentieth Century Practice, vol. x. 

36 Epilepsy and other Convulsive Diseases, 1901. 

37 Trans. Med. Soc. State of Penna., 1897. 

8S Epilepsy, its Pathology and Treatment, 1890. 



NEUEOSES OCCASIONED BY EYE-STKAIST 795 

that exceptionally the so-called reflex and idiopathic epileptic convul- 
sions may be cured by correction of ocular anomalies. 

Treatment. — Ocular examination conducted with patience, ability, 
and thoroughness should be made in every case of epilepsy, including 
all its forms. In a large proportion, the result of ocular remedies, both 
on the violence and frequency of the seizures, will demonstrate only 
its negative value; in a smaller proportion, the seizures will be per- 
manently arrested. It would seem to the writer that a distinction be- 
tween epilepsy, epileptiform convulsions, petit mal and grand mal need 
not be drawn when we are face to face with the problem, What shall 
we do with this case of convulsions % The prognosis of the affection is 
universally admitted to be unfavorable, and why should we decline to 
investigate and remove all possible sources of peripheral irritation, 
whether the symptoms are said to belong technically to the so-called 
" true" epilepsy or are only epileptiform in character ? The therapy of 
drugs has failed to cure. The bromide treatment, that most generally 
resorted to, is admittedly palliative only. By dulling the cerebral 
forces, outbreaks or crises of cerebral activity are held in abeyance. 
The advocates of the bromide treatment recognize its value in reliev- 
ing symptoms, but do not claim for it curative power. Brain surgery 
accomplishes a little. Da Costa says that 4 per cent, of all epileptics 
are cured by operation on the skull. The removal of reflex irritation 
arising from atrophied eyeballs, nasal disease, old cicatrices, degen- 
erated testes, and other lesions, has been followed by relief of the con- 
vulsions, and, if well authenticated reports of cure by eye treatment 
are to be credited, a knowledge of the ocular conditions will suggest 
methods of treatment that we cannot afford to neglect. 

The examination of the eye includes, first, refraction, and, second, 
the extraocular muscles. 

1. Refraction. — (For detailed method of diagnosis and treatment of 
refraction errors, the reader is referred to one of the text-books on that 
subject.) (a) Hyperopia and hyperopic astigmatism. Under full 
paralysis of accommodation in individuals less than forty-five years 
old the total error is determined, and as near a full correction as will 
be tolerated, ordered, and worn constantly. Thus, the unusual exer- 
cise of accommodation, and the undue stimulation of the extraocular 
muscles dependent upon it, are removed ; the congestion of the uveal 
tract so commonly associated with accommodative asthenopia, reduced ; 
the circulation of blood and lymph restored to the normal and 
the excessive stimulation of the ciliary nerves, and consequent over- 
action of the other branches of the third nerve, relieved. In those 



796 THE EYE AND NERVOUS SYSTEM 

cases in which the accommodation conceals a latent error of refraction, 
suggested by the lack of harmony between the degree of refraction as 
determined by the ophthalmoscope, or ophthalmometer, or a difference 
of refraction between the near and far points, full paralysis of the 
ciliary muscle should be obtained by stronger solutions of the cyclo- 
plegic and its continued application. By these means the eye is fully 
rested, the degree of the true static error determined, and the causes, 
resident in the ocular tissues or the result of overuse, which con- 
tribute to the production of reflex symptoms, eliminated. 

(b) Myopia and myopic astigmatism. The influence of increased 
refractive power, in causing reflex phenomena, has already been alluded 
to. By spasm of the accommodation, by the unconscious non-use of 
the accommodation or the disruption of the relation of accommodation 
and convergence, local and constitutional symptoms of eye-strain may 
be produced. Myopia is, however, seldom named as a cause. As a 
rule myopic patients do not complain of asthenopia or other evidence 
of eye-strain, but only of indistinct distant vision. There is a gradual 
increasing tendency to divergence of the visual axes which is to be con- 
trolled, and its further development into an actual divergence prevented, 
by the muscles of convergence. The maintenance of binocular single vis- 
ion by the ever-diminishing power of convergence, leads either to the 
reflex neuroses in neurotic patients, or to dissociation of the eyes in 
the act of vision. The real defect, as in hyperopia, can be detected only 
through cycloplegia, but unlike hyperopia, the correction selected with 
mydriasis is apt to be stronger than the actual degree of myopia. Post- 
mydriatic examination and the ordering of glasses after complete return 
of the accommodation, in order that its power may be measured and 
allowance made when advisable, is essential to a practical and useful 
correction. In all cases, the examination should be repeated, at in- 
tervals of one or two years, and changes in the refraction followed by 
corresponding changes in the glasses. 

(c) Presbyopia. Comparatively few cases of neuroses are to be 
attributed to the strain of the eyes from failure of the accommodation 
due to stiffness or loss of elasticity of the lens, or to the failure of con- 
tractility of the ciliary muscle. Yet such cases have been reported, and 
marked amelioration of distressing symptoms has followed the use of 
carefully selected glasses. The strain arises from the expenditure of 
nervous energy in the effort to focus the eyes for small objects at 33 cm. 
distance, and because of the excessive innervation to, and response of, 
the muscles of convergence without an equivalent response of the ciliary 
muscles. The strain may induce, in individuals predisposed to nervous 






LEUKOSES OCCASIONED BY EYE-STEAIX 797 

affections, the identical phenomena that are witnessed in younger per- 
sons who suffer from eve-strain. The correction of presbyopia is a 
simple matter. Having learned the refraction of the eye, glasses should 
be given which supply the deficiency of accommodation so that the 
patient will read without discomfort at 33 cm. 

2. The Extraocular Muscles.. — In all cases of insufficiency of the 
extraocular muscles, readily determinable by the usual tests, and by the ' 
relations which the findings of the tests bear to the relative strengths of 
adduction and abduction, the true static refraction must be learned and 
the correction of it worn long enough both to make certain that the 
symptoms are not due to ametropia and to exert its beneficent influence 
on the muscular tensions. In persons too young to wear glasses, long 
continued atropinization may be effective. Such preliminary treat- 
ment of muscular anomalies before resorting to prisms or operations is 
imperative. 

(a) E soph or ia is a tendency to cross the visual axes. The rela- 
tively weak divergent power is insufficient to maintain binocular single 
vision for all distances without undue stimulation, and in order to 
obtain relief, treatment should be directed to the reduction of the 
demand on divergence. This may be accomplished by (1) the internal 
exhibition of nerve sedatives and simultaneous exercise of the muscles 
of abduction by prisms and the stereoscope, or the amblyoscope of 
Worth; and (2) by prisms, bases out, the degree of the prism to be 
determined by the amount of esophoria. The prisms may be incorpor- 
ated into the ametropic correction, or the latter may be decentred so 
that it has prismatic action, or they may be worn separately. Although 
this method is occasionally serviceable, it is open to the objection that 
it can only be useful by allowing the visual axes to turn inward — 
esotropia. It is only a crutch for the disabled muscles, and is not instru- 
mental in restoring the muscles to equilibrium. Moreover, the limit of 
the usefulness of prisms is soon reached. Esophoria develops under their 
action, either by uncovering a part of the defect hitherto latent, or by 
actually creating a greater defect. Notwithstanding this theoretic 
objection, cases that resist cure by complete and continued rest of ac- 
commodation, sedatives and abduction exercise, should wear prisms 
before resorting to the final means of cure. (3) Operation. Shall 
tenotomy or advancement be chosen ? Esophoria, dependent upon hy- 
peropic refraction, is not primarily induced by weakness of divergence, 
but by excess of convergence, a physiologic sequence of the inordinate 
action of the accommodation. Hence tenotomy of one or both of the 
interni is the logical procedure. The operation should be performed 



798 THE EYE AND NERVOUS SYSTEM 

with great care and frequently interrupted to note, by testing with the 
Maddox rod or other means, the effect of work already performed. 
Free division of the tendon and adjacent structures is unjustifiable. 
Partial or graduated tenotomy only, as recommended by Stevens and 
Ranney, is the proper operation. These surgeons state that equilibrium 
can seldom be secured after a single operation, and claim that, should 
more defect be uncovered as time goes on, a second, third, or more 
graduated tenotomies should be performed, the necessity for the subse- 
quent operations being indicated by a return of the symptoms. 

(b) Exophoria is a tendency of the visual lines outward. The cor- 
rection of ametropia, the use of nerve stimulants in large doses, prism 
exercise of the weakened convergence, the restriction of the time de- 
voted to the near use of the eyes, and other hygienic measures referred 
to under esophoria, will, in the majority of cases of exophoria, obviate 
the necessity for operation. Should, however, the symptoms resist this 
treatment and the wearing of prisms, advancement or resection of one 
or both interni should be preferred to tenotomy of the externi. Thus, 
deficient adduction may be strengthened and made to compensate for 
the relatively stronger abduction. 

(c) Hyperphoria is a tendency of the visual axes to separate ver- 
tically, or of one visual axis to assume a plane higher than that of its 
fellow. This is the most frequent of the anomalies of the muscles of 
the eye to cause reflex neuroses, and to Stevens is due the credit of 
scientifically and practically elaborating the causal relations, and of 
bringing the subject impressively to the notice of the profession. As 
in the other muscular anomalies, this is dependent in the large ma- 
jority of cases upon errors of refraction. The principles of treatment 
are essentially those of other defects of co-ordination. 

CHOREA. 

Involuntary tonic or clonic contraction of a muscle, or of groups of 
muscles, is a symptom of an organic disturbance of the cerebrospinal 
system, or a reflex perversion of function of one or more of the organs 
of the body. The former, or " true" chorea, is, according to some 
authorities, an acute infectious disorder, variable in its limitations, 
nature and course, according to the character of the basic disease. The 
latter " habit," reflex or symptomatic chorea, is a purely functional 
affection, the development of a peripheral irritation. 

The word chorea has been interchangeably used by writers in de- 
scribing true chorea and habit chorea, affections that are essentially 
different in their etiology, prognosis, and treatment. In consequence 



LEUKOSES OCCASIONED BY EYE-STRAIN 799 

of this vagueness of definition, much confusion has arisen as to the 
true significance of chorea, and unprofitable controversies, more or less 
bitter, have been waged in medical journals. 

The relation of eye-strain to the causation of true chorea is prac- 
tically denied by authors of wide experience — men whose opinions are 
worthy of the greatest consideration. Thus, Lees, 39 in an accurate and 
presumably full description of the pathology and treatment of this 
disease, makes no mention of eye-strain and this possible cause is not 
alluded to by the speakers who discussed his paper. M. Allen Starr 
scientifically studied 1400 cases treated in the Yanderbilt clinic, and 
KrafTt-Ebing 200 cases, 40 without finding a single subject in which the 
cause could be attributed to eye-strain. Aldrich, 41 in an impartial 
study of the subject, criticises Stevens's and Itanney's reported cases 
of the cure of chorea by eye treatment, by stating that their cases were 
habit spasm, or possibly hysterical chorea, and not one case was true 
chorea. " It was merely a question of mistaken diagnosis." Baker's 
report of his cases 42 was also criticised by Aldrich 43 in these words : 
" He did not believe that the winking and grimacing of children as 
described by Baker to have been relieved by glasses was chorea, and 
that chorea proper could not be caused by eye-strain since it was un- 
doubtedly an acute specific nervous affection." Starr further says 
" while it is possible that local twitching of the muscles of the eyes or 
face or neck may be produced by eye-strain or irritation of the naso- 
pharynx, true chorea is never, in my opinion, produced by these causes, 
and treatment directed to the relief of the anomalies of the eye muscles 
is useless." 

The chorea of eye-strain is essentially that described years ago by 
S. Weir Mitchell as " habit chorea," and more recently by Osier 44 as 
" simple tic" or habit spasm. According to the latter authority, it is 
to be carefully differentiated from chorea with superadded psychical 
phenomena, and from complex and co-ordinated chorea. Further, as 
Wilbrand and Saenger have pointed out, 45 habit chorea should not be 
confounded with or mistaken for the reflex chorea, or choreic move- 
ments, of the orbicularis palpebrarum (blepharospasm), of the frontalic 
and the corrugator supercilii, which are dependent upon irritative or- 

39 British Medical Journal, 1903, 11. 

40 Festschrift in honor of A. Jacobi. 
"Arch. Pediat., 1903, xx. 
"American Medicine, March 14, 1903. 

43 American Medicine, May 9, 1903. 

44 Arch. Ped., January, 1897. 
48 Die Neuroloade des Auges. 



800 THE EYE AKD NERVOUS SYSTEM 

ganic lesions of the cortical centre, nucleus, or of the seventh, fifth, or 
optic nerve trunks. 

Choreic movements, the result of eye-strain, involve primarily the 
muscles of the lids, secondarily, those of the face and neck, and in ex- 
ceptional cases, distant groups of muscles may be invaded. The extent 
of the spread of the movement and its violence, are, in a measure, 
independent of the source of the strain, whether it be due to an error 
of refraction or to a muscular anomaly. The separation of these two 
etiological factors, is, in a majority of cases, not practicable. As has 
already been shown, muscular anomalies rarely exist except in connec- 
tion with, or dependent upon, errors of refraction, which are rarely 
found in association with perfect muscular balance. The character 
and intensity of the reflex phenomena of eye-strain are determined less 
by the special ocular error than by the impressionability of the nervous 
system, the age, occupation, and environment of the individual. 

The credit of the discovery that astigmatic and other optical errors 
may be responsible for reflex nervous phenomena belongs to William 
Thomson, who, in 1879, published 46 a clinical lecture delivered in the 
Jefferson Medical College Hospital, in which he says, " More than ten 
years ago I became aware that persons who consulted me for imperfect 
sight presented symptoms not fairly described in the text-books under 
the general head of asthenopia and which were indicative of some 
serious disturbance of the nerve centres." Among other symptoms he 
mentions insomnia, loss of appetite, a fear of some impending disaster, 
apoplexy or epilepsy, general nervous prostration, choreic twitching of 
the muscles of the head or face. (Italics mine.) Finding these persons 
astigmatic and seeing that their symptoms yielded to the supporting 
effect of properly selected cylindric glasses, the attention of S. Weir 
Mitchell was called to this aspect of these sufferers, and to the latter dis- 
tinguished physician Thomson's discovery seemed of sufficient impor- 
tance to bring it to the notice of the general profession in a paper in the 
Medical and Surgical Reporter, 1874, and the American Journal of the 
Medical Sciences, 1876. 

During the thirty years that have elapsed since Thomson's dis- 
covery and pioneer work in this great department of ophthalmology, 
tens of thousands of patients have been successfully treated for nervous 
disorders by remedies directed only to a readjustment of the focussing 
and co-ordinating apparatus of the eyes. Stevens's study of the ocular 
musculature and his philosophical deductions and practical application 



Medical News and Library, June, 1897. 



NEUROSES OCCASIONED BY EYE-STRAIX 801 

of tliem to the correction of muscular anomalies 4T marks another epoch- 
making period in this branch of science. These men have been followed 
by a host of workers along the same lines, who have reiterated with 
increasing emphasis the importance of eye-strain as a factor in the 
causation of functional nervous diseases. 

Facial chorea, while not one of the common symptoms of eye-strain, 
is yet so frequently observed that oculists accept its presence as evidence 
of disturbed ocular function. Its only resemblance to true chorea con- 
sists in an involuntary contraction of voluntary muscles. Its appear- 
ance, unlike that of true chorea, is independent of season or age of the 
individual. It has a distinct relation to school life, in the young, and to 
certain occupations, because, in the acquisition of education, or study of 
a profession, as in other pursuits of life, the defective eyes cannot 
respond to the demand upon them without strain. The child is taken 
from school, the professional man from his books, the clerk from his 
ledger, and a cure results — a cure too likely to be credited to outdoor 
life, change of environment, rest from brain-fag, etc. Rest, recreation, 
exercise, tonics and the like, are of distinct value ; but, on the other 
hand, too much importance should not be attached to their influence. 
Resumption of work means return of symptoms. The remedies are of 
only temporary benefit. The fault lies, not in brain-fag, too much 
work, too much reading, too much indoor life, but in the use of the 
eyes under adverse conditions. Before reaching the decision that the 
patient is " run down" and needs winding up by the rest treatment or 
a course of tonics, or other hygienic measures, examination of the eyes 
should be made, in order to determine .whether an optical error or func- 
tional derangements of the muscles may be responsible. Wood's 
advice, 48 /' Especially are eye-strain and nasal difficulties apt to cause, in 
childhood, persistent chorea, and it is, therefore, essential in every case 
which resists treatment to thoroughly examine these organs," might be 
advantageously modified into a recommendation that the ocular and 
nasal possible causes be excluded before treatment directed to other 
organs be tried. 

Eye treatment is, in a sense, experimental. It is quite impossible 
to predict with certainty in any case that it will be efficacious. It is, 
however, reasonable and logical to assume from knowledge based on 
previous experience, or acquired through reading, that the ocular defect 
found in any given case is the source of the trouble, or in the absence 
of a defect that the ocular apparatus is not a causative factor. Wearing 

47 Arch. Ophthal., 1884. 

48 Pepper's Amer. Text-Book. 

51 



802 THE EYE AND NEKVOUS SYSTEM 

of a needed glass or the correction of a muscular error will soon decide 
the etiology. A large proportion of choreic patients have hyperopia 
and astigmatism, but 95 per cent, of all individuals have an error of 
refraction; therefore, it would appear that frequency of ametropia in 
choreics has little or no bearing on the relation of cause and effect. 
Cheney 49 examined 23 cases of chorea, and found emmetropia 2, 
hyperopia 15, hyperopic astigmatism 3, myopia 1, myopic astigma- 
tism 1, unknown 1, esophoria 8. His conclusion was that " patients 
frequently recover while undergoing no treatment whatever, but the 
marked improvement or rapid recovery following the correction of re- 
fractive or muscular errors in some of these cases makes it seem more 
than probable that the ocular defects were the exciting cause of the 
disease." The same writer reports the case of a boy, eight years of age, 
who, since his fifth year, had never been free from chorea, although the 
twitchings were slight at all times. The patient complained of head- 
aches for a number of years. Two months previous to Dr. Cheney's 
examination it was noticed that the left eye turned inward; inci- 
dentally the twitchings became considerably aggravated, the movements 
involving both the upper and lower extremities, neck, and face. An 
examination of the eyes under atropia revealed, in addition to the con- 
vergent strabismus, hyperopia of 1.50 D. in both eyes. A cure was 
effected by correcting the refractive error and tenotomizing the internal 
rectus. De Schweinitz examined 50 cases of chorea in the Orthopedic 
Hospital and Infirmary for Nervous Diseases, and in 77 per cent, of 
these patients hyperopia and hyperopic astigmatism were the pre- 
dominating condition of the refraction, but " evidence seems lacking 
that the refraction error is the basal cause of the chorea." Of 118 
cases examined by Stevens in his private practice, all but 5 had .defective 
refraction, and a considerable number muscular insufficiencies. 50 

Roosa 51 says that of 118 cases of chorea, 78 had hyperopia, 13 
had hyperopic astigmatism, 5' had mixed astigmatism, 6 were myopic, 
and 11 had myopic astigmatism. 

These figures, and others of like import, simply show that refrac- 
tion errors are extremely common among choreics, but they do not prove 
that ametropia is the primary causative factor in producing chorea. 
It is, however, reasonable to suppose that accommodative or extrinsic 
muscular strain, like irritation in any other part of the body, may 



49 Boston Medical and Surgical Journal, February 20, 1890. 

50 The reader is referred to the remarkable array of case histories in his book 
on Functional Nervous Diseases. 

51 Transactions of the New York Academy of Medicine. 



LEUKOSES OCCASIONED BY EYE-STRAIN 803 

aggravate the symptoms of chorea or any other nervous affection. Eye- 
strain is a waste of nerve force and escape of energy, a consumption of 
power so easily prevented, so amenable to treatment, that whenever even 
a suspicion of its presence exists an examination should be demanded. 

VERTIGO. 

Vertigo, subjective or objective, is a symptom of many diseases, 
organic and of known pathology, and of functional affections which 
have no recognizable or defined lesions. It is found, for instance, in 
disease of the inner ear, irritative intracranial affections, disease of the 
heart and blood-vessels, of the blood (toxaemia), and other affections by 
which equilibration is temporarily or permanently destroyed. 

Vertigo may be reflex. Mills says, " Irritation may be reflected to 
the apparatus of equilibration from almost any part of the body, near 
or far. Reflex vertigos are termed ocular, aural, nasal, pharangeal, 
gastric, gastro-intestinal hepatic, uterine, or ovarian, according to the 
organ which seems to be the fountain-head, but if they are truly reflex 
disorders the mechanism of their production is practically the same 
in all cases, — namely, a disturbance of equilibrium produced by the 
reflection of an abnormal stimulus upon a more or less non-resisting 
equilibratory apparatus." It will be seen that the presence of vertigo 
or dizziness is of little value in determining the nature of the under- 
lying affection. The diagnosis is, necessarily, often one of exclusion. 
The appearance of vertigo incidently with the performance of the func- 
tion of one or more organs of the body will, however, often indicate its 
probable origin. 

Ocular vertigo is the result of causes identical with those that are 
active in giving rise to other symptoms of irritation or exhaustion of 
the nervous system, — namely, accommodative strain and muscular insuf- 
ficiencies. Any uncorrected error of refraction, or presbyopia, or hetero- 
phoria in an individual predisposed to nervous affections may be respon- 
sible. Vertigo occurs in about 12 per cent, of all cases of asthenopia, 
including both accommodative and muscular, 52 in which no disease of 
the visual apparatus can be shown. It is common also in those that 
have asthenopia from wearing inaccurately adapted lenses, either in 
the strength of the lens or in the adjustment of the axis of the cylinder. 

That vertigo may be a symptom of eye-strain was shown by Thom- 
son and later by the joint papers of Thomson and Weir Mitchell. 
Their statements have been verified in thousands of cases, and are 
being daily shown to be true in the routine work of the oculist. 

"Marlow, New York Medical Journal, July 13, 1897, gives 29 per cent. 



804 THE EYE AKD NERVOUS SYSTEM 

Ocular vertigo is, with few exceptions, associated with, or conse- 
quent uj)on, the use of the eyes for near work. It is purely subjective, 
the patient becoming dizzy and feeling as though he would fall. Ob- 
jective vertigo, or the apparent movement into unreal or unnatural 
positions and relations of stationary objects, is rare. Ocular vertigo is 
momentary, disappearing with the cessation of the use of the eyes and 
recurring with resumption of work. The relation of its oncoming to 
the performance of close work depends, as in other forms of asthenopia, 
upon the relative strength of the accommodation and of the extrinsic 
muscles, as compared to the degree of the ocular defect. The condition 
of the general health has also much to do with the advent and fre- 
quency of the attacks of dizziness. The attacks recur with greater 
frequency in those who are weakened from disease or overwork, who 
lead sedentary lives, who are confined many consecutive hours to 
their desk or sewing-machine, or who have some other affection 
tban asthenopia. And conversely, the attacks are less severe and fre- 
quent in the healthy and well-fed and among those who observe the 
rules of hygiene. 

Dizziness seldom exists alone as a symptom of eye-strain, but is 
associated with local asthenopia and other reflexes, particularly nausea. 
The gastric disturbance may be secondary to the vertigo or primarily 
induced by the cause of the vertigo. The combination of dizziness, 
nausea, and vomiting is not accidental, but an intimation of the asso- 
ciation of eye-strain with functional stomach disorder. All these symp- 
toms may be, of course, the result of eye-strain alone, particularly when 
they occur with, or after, use of the eyes. 

Immediately preceding and during the attacks of vertigo, the con- 
dition of the cerebral circulation is doubtful. It is probably not the 
same in all sufferers, and not always the same in one individual. 
Whether it be one of anaemia or hyperemia, the circulatory disturbance 
is momentary and probably limited to an extremely small central area. 
Upon removal of the cause or cessation of the use of the eyes, the dizzi- 
ness is allayed, because the cerebral disturbance, whatever it may be, 
is removed. The effect of repeated irritation of the centre of equilibra- 
tion is deleterious, and while for a time the affection may be functional, 
constant repetition must lead to organic change with permanent loss 
of normal function and deterioration of the mental faculties. 

Diagnosis. — The characteristic features of ocular vertigo are its 
subjectiveness, transitoriness, and association with the act of vision. 
In order to test its ocular origin, the patient should abstain from all 
sources of eye-strain, either by voluntarily giving up the use of the 



LEUKOSES OCCASIONED BY EYE-STRAIN 805 

eyes or by induced paralysis of accommodation. Should the vertigo 
persist, the cause must be looked for elsewhere than in the visual appa- 
ratus. The subsidence of the vertigo, on the other hand, indicates that 
it is of ocular origin. Vertigo, dependent upon errors of refraction 
and functional anomalies of the muscles, is, strictly speaking, ocular 
vertigo, and should not be confounded with the vertigo of organic dis- 
ease of the ocular nerves, in which the lesion is orbital or of the cerebro- 
spinal system. For example, hemianopsia, limited fields of vision, 
diplopia, the apparent distortion of objects by imperfect retinal images, 
hallucination of vision, etc., frequently are associated with vertigo. 
Vertigo of brain tumor, in which the optic or other ocular nerves are 
involved, is also not to be classified as ocular vertigo. 

Treatment. — Correction of errors of refraction and the restoration 
to balance of the extrinsic muscles are imperative. Rest of the eyes 
by abstinence from the usual occupations, tonics, and other general 
measures, will give temporary relief, but permanent cure can be estab- 
lished only by removal of the defect of the visual apparatus which is 
the cause of the eye-strain and vertigo. 

A few illustrative examples from the journals and from my own 
histories are appended. 

Colburn 53 quotes the case of a farmer who suffered from severe 
headaches, vertigo, and nausea. He was examined under atropia, and 
was found to have a hypermetropic astigmatism in each eye, besides a 
hyperphoria of one degree and a manifest esophoria of eight degrees 
in the right eye. He was given his correction and advised to return 
if the symptoms did not abate. Four and a half years later he appeared, 
not having complained of any discomfort until recently. No change 
was found necessary in his glasses. The hyperphoria had increased, 
however, to four and a half degrees, but the esophoria remained the 
same. The right superior rectus was tenotomized and the inferior of 
the same eye advanced, the result being that a hyperphoria of one degree 
remained and the esophoria almost entirely disappeared. He was seen 
two months later, when he stated that the symptoms from which he 
suffered had not returned. 

The same author 54 gives details of four classes of intractable asthen- 
opic headaches, with insomnia, vertigo, indigestion, and, in one case, 
petit mal, persisting in spite of careful refraction. Correction of mus- 
cular anomalies gave relief in four cases. 

Eulton (in Northwestern Lancet, 1000) reports the case of a busi- 

53 Clinical Lectures on Diseases of the Eye. 

54 Clinical Review, May, 1901. 



806 THE EYE AND NERVOUS SYSTEM 

ness man who suffered from almost constant vertigo. For two years 
he was treated at sanitariums in America and Europe without obtaining 
relief. Examination showed a heterophoria. A tenotomy of the in- 
ferior rectus was done, with the result that all symptoms of vertigo 
completely disappeared, and, up to one year after the operation, had 
not returned. 

Carter 55 reports the case of a young man, the son of a wealthy 
banker, who was obliged to relinquish his studies at Oxford because of 
his inability to read without incurring diplopia, followed by vertigo, 
and, if continued efforts were made to read, by sickness, palpitation of 
the heart, and intense headache. Both eyes were found to be myopic, 
but otherwise the media and fundi were normal. He was refracted 
and suitable glasses adjusted, with the result that all symptoms from 
which he had suffered disappeared almost immediately and had not 
returned when seen about three months later. 

Case 1. Mrs. T., aged 29 years. Complained of attacks of ver- 
tigo, lasting thirty minutes, followed by severe headache and occasion- 
ally by vomiting. The dizziness commenced at 15 years of age, 
and recurred every week or twice a week for the past 12 years. The 
attacks were attended with numbness of the fingers. V=G/6. R. hyper- 
phoria %°, esophoria y% \ She was relieved by full correction of 
HAs. 

Case 2. Mrs. T., 48 years, momentary dizziness while working 
as a weaver or while walking. The trouble commenced simultaneously 
with the wearing of glasses for the correction of compound HAs., and 
presbyopia. Cure was effected by addition of the prismatic correction 
of 2° of hyperphoria. 

Case 3. Mrs. K., 46, nearly constant headache ; efforts to see near 
objects invariably induced vertigo, nausea, and vomiting. Correction 
of high-grade mixed astigmatism and presbyopia gave entire relief 
from her distressing symptoms. 

Case 4. Mrs. I., 27. Could not read half an hour without head- 
ache and dizziness. She had always considered herself nervous and 
excitable. Headache and vertigo were cured by 25 D. less than full 
correction, which under cyclopegia was found to be 

It. + .75 o + .25 ax. 15° 
L. + .50 o + 1. ax. 165° 

Case 5. Mary F., 11. Neuralgia in left side of face and dizziness, 
aggravated by near use of the eyes. Print seemed to grow small and 

53 Transactions of the Clinical Society of London, vol. viii. 



LEUKOSES OCCASIONED BY EYE-STKAIJST 807 

recede. She also suffered from constipation, pain in back of neck and 
across trie shoulders. These symptoms were cured by: 

E. + 2.25 ax. 90° 

L. + .500 + 2.25 ax. 90° 

Multiplication of cases is unnecessary. It is universally admitted 
that vertigo may be a symptom of ocular strain, and it is cured by 
lenses to correct refraction and presbyopia, by prisms, and by opera- 
tions designed to overcome abnormal tendencies of the ocular muscles. 
It is, however, interesting to note that practically identical ocular errors 
give rise to no reflexes in some patients, while in others they may cause 
vertigo, gastric disturbances, or a complexity of symptoms, which ap- 
parently have no connection with the use of the eyes. For example, 
May F., a sister of Case 5, had much the same error with entirely 
different symptoms. Her refraction was R. -j- 150 O + 1.50 ax. 90°; 
L. + 1C + 3 ax. 105°. She complained only of occasional head- 
ache and confusion of print in reading. Edward F., a brother, made 
no complaint at all except imperfect vision, and his defect was 

R. — .50 o + 450 ax. 100° 
L. — .50 o + 1 ax. 90° 

To revert to a principle, already mentioned, the optical or muscular 
defect does not decide the character of the reflexes. 

GASTRIC DISTURBANCES. 

The gastric symptoms of ocular strain are those of so-called 
functional stomach affections, — namely, flatulency, delayed digestion, 
hyperacidity, nausea and vomiting, and the natural sequence of indi- 
gestion — constipation. They manifest themselves in different in- 
tensity, according to the kind and degree of optical defect and accord- 
ing to the individual disposition. They may develop in persons whose 
digestive organs would be, under normal ocular conditions, in perfect 
functional activity, or in those whose digestive tract is disordered, as a 
result of other cause than ocular. They also occur as the result of 
disease of the digestive tract, and are aggravated by the use of the 
eyes. The history of their appearance, severity, continuance, and 
recovery corresponds in every particular to the history of other forms 
of asthenopic and reflex neuroses, appearing simultaneously with the 
commencement of close (or far) application of the eyes, and in a few 
minutes increase in intensity until the occupation is discontinued and 
the patients' eyes are put at rest. The symptoms persist during the 
period of eye-strain, and recover only after abstinence from work and 
possibly seclusion in a dark room. 



808 



THE EYE AND NEKVOUS SYSTEM 



Ophthalmic ganglion 
Carotid plexus 
Infraorbital 



Second division 
fifth nerve 



Meckel's ganglion 



Superior cervical 
ganglion 



Middle cervical ganglion 
Inferior cervical ganglion 



First division fifth nerve 

, First division third nerve 
First division fifth nerve 

Nucleus third nerve 

Nucleus fifth nerve 



Ganglion of the 
root 
Medulla of pneumogas- 

tric nerve 
First cervical nerve 
Second cervical nerve 



Spinal cord 



Pneumogastric nerve 




(Esophagus 



Fig. 1.— A schematic diagram of the cerebrospinal and sympathetic nerve connections between the 

eye and the stomach. 



NEUROSES OCCASIONED BY EYE-STRAIN 809 

The schematic anatomical explanation upon the opposite page 
(Fig. 1) of the origin of the gastric disturbances from irritation 
of the fifth pair of nerves helps to elucidate their ocular causes. It 
is, indeed, applicable to lesions occurring anywhere in the course of 
the fifth nerve. 

That indigestion may be due to eye-strain there can be no reason- 
able doubt. Too many authentic cases are on record to permit of scepti- 
cism. Unquestionably, hasty eating, imperfect mastication, ice-water, 
resumption of mental or physical work immediately after partaking 
of heavy meals, indifference to the laws of hygiene, and, in a word, too 
little thought of health, are contributory causes. Nerve energy that 
should be spent in stimulating the different organs of the body to per- 
form their functions is diverted to the visual apparatus. Focussing 
rays of light from near or distant objects and the adjustment of the 
musculature of both eyes for single binocular vision for more or less 
prolonged periods demand an excess of nerve force that cannot be 
spared without sacrificing the functions of other organs. The causative 
relation of eye-strain is seldom suspected. Treatment directed to the 
abdominal organs gives temporary benefit only. Permanent recovery 
ensues only after correction of all optical defect. 

The ocular factor in the etiology of gastric disorder has been insisted 
upon by oculists for many years. 

The following histories are not uncommon : In the article by Thom- 
son, 56 already alluded to, nausea is included in the description of 
asthenopia induced by astigmatism. 

M. X., lawyer, aged 30, complained of occasional blurring of dis- 
tant vision, photophobia, chronic conjunctivitis, and headache, all of 
which symptoms he attributes to indigestion. Under mydriasis the 
refraction was +2 O +1° ax. 90°. Correction of this defect cured 
the local asthenopia, headache, and indigestion. The patient had sub- 
mitted to medicinal treatment for indigestion for years, and was not 
a little surprised at the cure by glasses. Miss M. complains that she 
cannot use her eyes day or night without pain. Is nervous, irritable, 
and dyspeptic. After correction of HAs., muscular exercise by prisms, 
nerve tonics, and, finally, tenotomy of one of the superior recti muscles, 
she reported that she could read or sew several hours comfortably and 
felt herself " a new creature." 

Gould 57 gives the history of a case of flatulent dyspepsia of twenty 
years' standing cured by the application of proper lenses. The patient 

56 Medical News and Library. 

57 Medical and Surgical Reporter, Februaiy, 1889. 



810 THE EYE AKD KEKVOUS SYSTEM 

complained of certain ocular and cerebral troubles, asthenopia, diffi- 
culty in near work and the like, besides the gastric disorder, though, 
it was the latter which gave her the greatest cause for worry. Medi- 
cines never gave her any relief. There was absolutely no hysterical 
element in the case. The reflection of the ophthalmoscopic mirror into 
the eye caused such an instantaneous and frightful paroxysm of eruc- 
tation that, for fear that violent emesis was about to take place, the 
examination had to be deferred. It was highly interesting to note that 
the application of suitable lenses in a trial frame as effectually quieted 
the gastric volcano as the ophthalmoscopic mirror had caused it. 
Glasses were prescribed, with the result that the patient improved each 
day. She stated that she could not leave the glasses off a minute with- 
out gastric symptoms. That the condition was purely a reflex neurosis 
was clearly evident, because removal of the glasses from before her eyes 
while fixing an object caused the eructations to reappear like a flash, 
and again disappear when the glasses were put in place. Knapp says, 
in the treatment of so-called gastric troubles, flatulent and other dys- 
pepsias, indigestion, etc., the neutralization of errors of refraction and 
restoration of normal muscle balance yield such good results that oph- 
thalmogists are justified in constantly advising ocular treatment. 
Ayres 58 reports a case of a healthy, well-developed boy of 15 years, 
who complained of nausea when in school, in church, or in any assem- 
bly of persons. The sensation became so marked in school that he 
was obliged to discontinue his studies. Ophthalmoscopic examinations 
revealed a hyperopia of 1.5 D. in each eye. He was refracted and 
ordered a +1 D. to be worn constantly. The results were immediately 
manifest The nausea was at once relieved. Parker 59 says that while, 
as a general rule, the symptoms produced by refractive errors and ocular 
imbalance are varied, the headaches are characteristic. The migraine 
produced by refractive errors begins as frontal, extends to the top of the 
head, and finally settles in the back of the neck. He quotes several 
interesting cases: 

1. A man, aged 35, complained of being nauseated and of having 
severe headaches for two or three days each time he audited his books, 
which was every month. So severe were these attacks and so directly 
referable to his near work that he was obliged to refrain from excessive 
use of his eyes for the balance of the month. He had a low myopia 
for which he used his correction constantly. Muscle test showed one 
degree of exophoria. By orthopic exercises the divergence disappeared, 

58 Cincinnati Lancet Clinic. 

59 Physician and Surgeon, May, 1902. 



NEUROSES OCCASIONED BY EYE-STRAIX 811 

as did the symptoms, after six weeks of training. This patient has 
been under observation for seven years, and in that time he has been 
entirely free from his old attacks. 

2. A girl, aged 10, suffered daily from intermittent headaches, 
nausea, and vertigo for six years. Examination showed an exophoria 
of 9 ° ; although glasses were given to correct the astigmatism £>res- 
ent, and adduction was developed by exercise, the symptoms continued. 
A complete tenotomy of the external rectus reduced the muscle error to 
naught. It later went back two degrees, but no symptoms developed and 
she has had perfect comfort ever since the day of operation. 

GENERAL ASTHENOPIA. 

Asthenopia, painful use of the eyes, is common to all forms of 
refractive defects and muscular anomalies, and is, exceptionally, present 
in emmetropia and muscular equilibrium. One or the other of these 
conditions is necessary for its development, but, as a rule, both factors 
are coexistent, and in most asthenopics, — namely, a defect in the visual 
apparatus and an impressionable and responsive nervous system. The 
use of emmetropic eyes under unhygienic conditions of light, improper 
physical posture, unusual or unnatural direction of fixation in healthy 
individuals, will cause asthenopia, which assumes an exaggerated ex- 
pression in those who are infirm or predisposed to muscular weak- 
ness. 

Asthenopia is accommodative or muscular. (It may be both accom- 
modative and muscular.) In the former case the power of accommo- 
dation is unable to cope with the disadvantages of ametropic refraction ; 
in the latter the nerve stimulation to the extra-ocular muscles or the 
muscles themselves are defective. Both forms give rise to local and 
general symptoms which may be logically referred to ocular causes or 
which may be so independent of the use of the eyes that their ocular 
origin is only suspected after exclusion of other possible causes. 

The Acuity of Vision in Accommodative Asthenopia. — Lowered 
vision, or the inability to perceive and name letters or objects that are 
small enough to be included in the 5' visual a liable, in anv distance be- 

O O 7 i 

tween the near-point and the so-called infinity (6m.), is not always, 
and indeed in the majority of cases, associated with or a symptom of 
asthenopia. Its presence is, however, indicative that the visual appa- 
ratus is imperfectly functionating. The defect may lie in opacity of 
the media, in disease of the optic nerve, from its deep origin in the 
brain to its expansion at the fundus of the eye, or in an error of refrac- 
tion. Eliminating disease, as irrelevant to the subject of this chapter, 



812 THE EYE AND NERVOUS SYSTEM 

we are limited to a consideration of Hyperopia, Myopia, and Astig- 
matism. 

(a) Hyperopia (H). — The acuity of vision depends upon the de- 
gree of H., the power of accommodation, and the distance from the 
eye at which the test is made. Young persons in good health may 
conceal, by ciliary contraction, 2 to 3 D. of hyperopia for all distances. 
3 D. may be consistent with full acuity for far, but not for near. 
Higher hyperopia than 3 D. in the .great majority of persons, even the 
very young, means lowered vision, and is therefore one of the symp- 
toms of an optical defect. In advancing years the contractility of the 
ciliary muscle becomes reduced and the elasticity of the lens lessened. 
Latent H. becomes manifest H., and the acuity of vision appreciably de- 
clines. Between forty and forty-five years of age in Em. distant V. may 
remain full, but the near-point has receded, and full acuity for the ordi- 
nary reading distance is no longer present, or, if present, is maintained 
for a few minutes only. In H. the distant V. always suffers with the 
receding of the near-point. In myopic refraction, distant and near 
vision are practically unchanged by failure of accommodation. 

(b) Myopia (M). — It will be seen from these statements that the 
acuity of vision depends in part upon the distances at which the tests 
are made, and that no examination is complete unless the determination 
of the position of the near-point is included. Lowered acuity for near 
is suggestive of hyperopic refraction, although myopic astigmatism may 
also be a cause: In M. vision is always less than 6/6. The ability 
to see small objects, such as test types, at the distance at which they 
are seen by emmetropic eyes, the accommodation being abolished either 
pathologically or therapeutically, is not incompatible with myopia of 
low degree. Myopes, however, usually read at a distance nearer than 
the degree of M. would warrant, since the convergence necessary, 
in the associated function of both eyes, induces contraction of the 
ciliary muscles, and this, in turn, increases temporarily the M. Re- 
laxation of the accommodation may not be complete even for 6 M.,. 
particularly in individuals accustomed to long-continued near work. 
Hence M. will be apparently greater than the degree shown under 
complete cycloplegia. I have seen a remarkable illustration of this 
fact. A woman, aged 35, had binocular V = 6/60 corrected to 6/6 
by — 3 D. When she fixed with one eye and allowed the other to 
diverge, V. improved to 6/6 without a glass. 

(c) Astigmatism (As). — Lowered visual acuity is found in all 
grades of MAs. and HAs. of 1 D. or over. In spasm of accommodation, 
by which HAs. is apparently transformed into MAs., the acuity of vision 



NEUROSES OCCASIONED BY EYE-STRAIN 813 

is below the normal and is improved by a minus cylinder before cyclo- 
plegia, a plus cylinder during cycloplegia. In mixed As. the myopic 
meridian may be made more myopic and the hyperopic meridian less 
hyperopic by contraction of the muscle of accommodation. In either 
case the acuity of vision is reduced. 

Full acuity of vision in the distance is present in most cases of 
accommodative asthenopia. Therefore, the test so often made, to de- 
termine whether the eyes are at fault by test cards as 6 M., is misleading, 
and the exclusion of the eyes as a causative factor because V = 6/6 
is incorrect. Should, however, the combination exist of full visual 
acuity and muscular balance for all distances, it is probable that Em. 
is present and the symptoms of which the patient may complain are 
not of ocular origin. 

The visual acuity, therefore, is no criterion of the presence of 
accommodative asthenopia, since asthenopia may be a symptom either 
in normal or defective vision. It is claimed by some writers, how- 
ever, that asthenopia is more frequently present in the former than in 
the latter, and consequently is to be attributed to the constant effort 
of accommodation necessary to overcome the slighter degrees of ametro- 
pia by which vision is maintained at the normal standard. Yet it 
should be borne in mind that one additional factor enters into the 
eye-strain in individuals who have eyes so defective that the accommo- 
dation is powerless to entirely overcome the defect. The contraction 
of the ciliary muscle continues as persistently in the higher as in the 
lower defects, and the imperfect vision is only partly or not at all 
restored. The mental effect of the unclear retinal images should not 
be disregarded. The mind is erroneously informed of the actual size, 
form, and relation of objects, and is led to form wrong conclusions, 
the effects of which are far-reaching. The discovery of this uncon- 
sciously misguided attitude towards the world exerts a baneful influ- 
ence upon the entire system, tends to melancholy and depression of 
spirits, social exclusion, and helps towards the development of a mis- 
anthrope. 

Vision in Muscular Asthenopia. — The effect on vision of the de- 
grees of muscular defect is analogous to that of the degree of ametropia. 
Vision may be single (binocular) for -all distances and at all times ; 
it may be single ordinarily and blurred occasionally, when the blurring 
may be taken as evidence that the weaker set of muscles are momentarily 
incapable of maintaining the tension necessary to resist the opposing 
muscles. Simultaneous with the deviation of the visual lines, the object 
looked at becomes indistinct in outline or appears double. Vision may 



814 THE EYE AND NERVOUS SYSTEM 

be permanently double, indicating insuperable deviation from each 
other of the visual lines. Asthenopia of great severity usually accom- 
panies the first and second or latent forms of insufficiency and dis- 
turbed mental equilibrium, from the presence of the two images, is the 
common result of the third form. 

The Facial Expression in Asthenopia. — Stevens and others have 
called attention to the modification of the facial expression, induced 
by the conscious or unconscious participation of some of the facial mus- 
cles in the accommodative and muscular effort to secure clear and single 
images. The facial expression is determined in part by the ocular 
defect. Eor example, the myope by contraction of the orbicularis lessens 
the vertical diameter of the commissure, and by cutting off the light 
rays that enter the periphery of the pupil, the circles of diffusion that 
fall upon the retina are reduced in size. The contracting muscle also 
modifies the curvature of the cornea by pressure upon the vertical diam- 
eter. The same statement holds for myopic astigmatic when the axis 
# of the astigmatism is horizontal, or a few degrees removed from the 
horizontal. Should the axis be vertical, or nearly so, better vision is 
obtained by turning the head on its vertical axis. In heterophoria the 
muscles of the brow are contracted, lining the forehead with vertical 
or horizontal furrows. In heterotropia the orbicularis of one eye is 
maintained in a state of constant contraction in order to exclude that 
eye from the act of vision, or the head is rotated in its vertical or hori- 
zontal axis in the direction of the weaker set of muscles. Thus, in 
paralysis of the third nerve of the left eye, the eye is diverged, and 
the false image can be merged into the true by turning the face towards 
the right. 

:i Wrinkles," or " crows' feet," at the external angles of the com- 
missures are in many persons an indication of eye-strain. In exagger- 
ated cases of ametropia and muscle imbalance, the facial muscular con- 
tractions assume the proportion of deformities, and are suggestive of the 
expression of horror, fright, astonishment, or other emotion. These 
lines may become permanent, and are often misleading indications of 
character and disposition. 

Causes. — Errors of refraction and anomalies of the extraocular 
muscles have been alluded to. They are by far the most frequent causes 
of asthenopia 60 and affect a large proportion of the population of edu- 
cated communities. The causes are most active between the tenth and 



60 For correction of these defects and the treatment of accommodative and 
muscular asthenopia the reader is referred to special works on ophthalmology. 



LEUKOSES OCCASIONED BY EYE-STKAIN" 815 

fortieth years of life, although no time of life is exempt. Hale 61 says 
that muscular asthenopia usually has its origin in the brain, depending 
upon a disturbed innervation or possibly, in some cases, upon a weak- 
ness of the power of fusion. In a small percentage of cases asthenopia 
depends upon either the absolute or relative weakness of the adductors 
or obliques, or upon the position of their insertions into the sclera. His 
own observations lead to the belief that want of converging power is 
the chief cause of this form of asthenopia. Optical defects are of such 
vast importance to health and intellectual growth that it is incumbent 
upon every parent or guardian of youth to seek an examination of the 
eyes of those under their care. 

Reflex asthenopia is sufficiently common to deserve more attention 
than is accorded to it. Painful use of the eyes may be due to nasal 
obstruction, to dental caries, to throat and nose affections, and to the 
reflected irritation from disturbed function of the organs of the chest 
and abdomen. A familiar example is asthenopia from disease of the 
cavities accessory to the orbit, such as ethmoiditis, rhinitis, and frontal 
sinus affections, by which the circulation of the orbit and eye is ob- 
structed or the passage of the tears through the nasal duct prevented. 

Constitutional Asthenopia. — Painful use of the eyes may result from 
any physical or mental condition which tends to lessen the physical 
powers of resistance or to the induction of irritability or undue im- 
pressionability of the nervous system. Among the former causes are 
convalescence from illness, toxaemia, gout, rheumatism, exhaustive 
hemorrhages (as in childbirth), anaemia, diseases of the blood, sedentary 
life, and impure air. Among the latter are functional and organic 
derangements of the cerebrospinal system, resulting from disappointed 
ambition and desires, anxieties, and a want of proper relation between 
financial income and outgo. The ocular defect in constitutional asthe- 
nopia may be small or entirely absent. The symptoms depend less on the 
state of refraction or of the muscles, and more upon a deficient muscular 
and nerve force. The ciliary and extraocular muscles, like other 
muscles of the body, are unable to respond normally to the demand 
made upon them, and the nerve stimulation to the muscles, originating 
in the consciousness, is weaker than the will which governs it. The so- 
called " retinal" asthenopia may be regarded as another name for con- 
stitutional asthenopia. The limit of endurance of the retina to the 
impression of light rays and that of the optic nerve to conveying the 
retinal impulse to the cortical centres is reached in a comparatively 

61 Transactions of the American Ophthalmological Society, vol. vii. 



816 THE EYE AND NEKVOUS SYSTEM 

short time. The retina and nerve become easily fatigued and soon 
exhausted, giving rise to confusion of images and impaired mental 
conceptions. The exhaustion is a symptom of the combination of opti- 
cal errors and of constitutional weakness, and is common in nervous 
affections, independent of ocular error, such as neurasthenia and hys- 
teria. " The subjects break down easily and are unable to continue 
work, and have but little initiative." (Dand.) 

Symptoms. — Etymologically, asthenopia means weak sight. Oph- 
thalmologically, the word has come to signify painful sight. The symp- 
toms are local and reflex. Of the former, pain constitutes the principal 
phenomenon. It is felt in the eye, in the orbit, through the radiations 
of the first and second branches of the fifth nerve, and the first and 
second cervical. It follows immediately, or not for some minutes or 
hours after using the eyes, either for distance or in reading. It is slight 
or severe, sharp, neuralgic, or dull and boring, intermittent or constant, 
acute or chronic. It may have, apparently, no connection whatever 
with the use of the eyes or be originated or increased by reading or 
other near work. Occasionally ciliary tenderness is present, but in 
most cases the pain is temporarily lessened or wholly relieved by ex- 
ternal pressure upon the eye-balls. Blurring of letters in reading or 
the needle in sewing, or confusion of points and lines in any near work 
is due to transiently relaxed accommodation to momentary suspension 
of convergence or to exhaustion of retinal energy. The time of the 
oncoming of asthenopia after commencement of the near use of the 
eyes is, like other forms of asthenopia, dependent upon the power of 
accommodation, the relative strengths of the extrinsic muscles, and the 
tone of the nervous system. Hyperemia of the conjunctiva and lach- 
rymation may be associated with pain or occur independently when 
the eyes are subjected to strain in the effort for the accurate definition 
of images, particularly if long continued. 

Reflex Symptoms. — Headache is one of the most constant and aggra- 
vated symptoms of ocular strain in individuals under 40 years of age. 
It is a reflex pain of muscular effort and arises from excessive or pro- 
longed contraction of the ciliary muscle, from a disturbance of the 
normal relation of accommodation to convergence, or from imbalance 
of the external ocular muscles, intrinsic in the muscles themselves, or 
in their innervation and possibly independent of the refraction of the 
eye. 

Location. — Some authors 62 assign definite portions of the head as 



62 Marlow, C. A. Wood. 



NEUROSES OCCASIONED BY EYE-STRAIX 817 

the seat of ocular headache — frontal or temporal, for instance — in con- 
tradistinction to the location of headache from other causes. Wood 
has stated the order of frequency to be supraorbital, deep orbital, f ronto- 
occipital, and temporal. 

The exceptions to definite localization are so numerous that but 
little diagnostic value attaches to the location designated by the patient. 
In one hundred successive cases of accommodative or muscular strain 
under my care sixty-eight patients complained of headache, classified 
under the descriptive term " general," in twenty cases the pain was 
frontal, two temporal, and in ten occipital. The disproportionately 
large number of general is partly the result of inaccuracy of locali- 
zation, observation, and expression on the part of the patient, and to 
the dissemination of the pain throughout the head, from its origin 
within the orbit. The distribution of pain must be along the sensitive 
nerves of the head, which in their course or termination are subjected 
to irritation. Hence the pain felt outside of the orbit is reflex. Orbital 
pain, that arising from muscular tension or irritation of orbital nerves, 
might readily be referred to the temporal and frontal regions of the 
head through the distribution of the fifth pair of nerves. The exhausted 
ciliary muscle, through sympathetic fibres or directly, causes an irregu- 
lar discharge in the region of the third nerve nucleus or an irritation 
of the necleus itself. Occipital pain, often a symptom of muscular 
anomaly and of uncorrected astigmatism, is referred, not to the scalp, 
but to underlying tissues, and is possibly explained by the presence of 
localized congestion of the vessels and perivascular tissues at the base 
of the brain, induced by the effort to maintain balance of the extra- 
ocular muscles or by irritation of the third nerve communicated to the 
sensitive nerves of the meninges. 

Wilder 63 believes the vessels of the brain and meninges are dilated. 
Ocular headaches are due, according to Wilder ( 1 ) to exposure to bright 
light in cases of hypersensitiveness or irritation of the retina, which 
may be a precursor of retinitis, and to the frequent presence of venous 
dilatation; (2) to strain of accommodation in near use and in pro- 
longed distant vision, giving rise to " academy headache," as it is 
termed in London; (3) to muscular insufficiency. 

Frequency. — To compute the relation and frequency of ocular head- 
aches to headaches due to other causes would be an interesting but an 
almost impossible task. 64 To learn the frequency of headache as a 



63 Ocular Headaches, J. A. M. A., November 10. 1898. 

64 C. A. Wood states that 40 per cent, of all headaches, and 80 per cent, of 
frontal headaches, are due to eye-strain. 

52 



818 THE EYE AND NEKVOUS SYSTEM 

symptom of ocular disorder is only to review the case-book of any 
oculist in active practice. My own records show 85 per cent, in per- 
sons under 45 years of age who are free from recognizable organic 
disease of every description, ocular or general. Hospital records 
would of course show a less percentage since hospital cases include 
more diseased conditions. Zimmerman's statistics of all classes of 
headaches caused by ocular defects 65 show 67.8 per cent, in private 
practice and 74.9 in hospital cases. Zimmerman explains the higher 
ratio by the large number among hospital cases who must use their eyes 
for close work many hours in the day, such as weavers, woollen-mill 
workers, etc. Mnety-six of his patients had monocular vision only 
(strabismus or enucleation of one eye), and of these forty had headaches. 
Diagnosis. — The headache is usually induced by the use of the 
eyes, both in near work and in prolonged effort to see clearly in the 
distance, and consequently the connection of the use of the eyes with 
the development of the exacerbation of the pain is to be determined. 
It must not be forgotten, however, that some individuals, particularly 
with chronic headache, trace no connection between reading or other 
close work and the headache. In such cases it is practically impossible 
to ascribe the cause to the eyes until after the therapeutic or optical 
test is applied. The former consists of the instillation of a cycloplegic 
for a few days or the abolition of the accommodation. The latter, of 
the continuous wearing of the correction of any error of refraction that 
may have been found under paralysis of accommodation. Yet it must 
be remembered that the presence of muscular anomalies of sufficient 
degree to become a factor in the causation of headache vitiates these 
tests, for it is not uncommon to find that headache persists or is in- 
creased because of the participation of the extraocular muscles in the 
visual act. This is shown in cases of functional internal hyperopic 
squint. For some days after the initiation of the use of a cycloplegic 
the degree of squint may be higher than before its use. The innerva- 
tion of the muscles of convergence is not relaxed because of the tem- 
porary failure of the ciliary muscle to respond to its innervation. The 
diagnosis is simplified by the discovery of ocular strain induced by 
hyperopia, myopia, or astigmatism, as shown by lowered acuity of 
vision, by the ophthalmoscope, ophthalmometer, retinoscope, and test 
lenses, or by imbalance of the ocular muscles, as revealed by the Maddox 
rod, cobalt glass and diplopia test, and the proportionately increased 
tension of the muscles of adduction and abduction or sursumduction. 

65 New York Medical Journal and Philadelphia Medical Journal, November 21, 
1903. 



NEUROSES OCCASIONED BY EYE-STRAIX 819 

In every case knowledge of the functional activity of other organs of 
the body contributes materially to the accuracy of the diagnosis. Or- 
ganic disease of the nervous system, abdominal organs, digestive tract, 
syphilis, gout, rheumatism, and other general affections should be 
excluded. 

Simulating ocular headaches more closely than the other varieties 
are those caused by nasal disease. Here the pain is frontal, more often 
dull than sharp. Disease of the nose is, in many cases, excluded by 
examination of its cavities. But Ewing 66 describes a form of head- 
ache of nasal origin, characterized by neuralgia of the nasal and eth- 
moid nerves and tenderness on pressure on the inner upper angle of 
the orbit of one side, rarely both, in which the inflammation is not 
confined to the frontal sinus. Rhinoscopic examination revealed in his 
case no signs of the trouble. The headache is dull, persistent, located 
in the frontal region and generally worse in the morning. The cause 
of the headache is referred to the eyes, because the pain is greatly 
aggravated by attempts at reading, and attacks of conjunctivitis and 
blepharitis are frequent. The diagnostic features are tenderness, lim- 
ited to one orbit or one nasal bone, and asthenopia. 

The near work includes all the ordinary occupations — reading, 
writing, sewing, weaving, reading music, and amusements, such as 
card-playing. Ocular headache is quite common among card-players 
w T ho have entered the presbyopic age, as the games are usually played 
by artificial light and for several consecutive hours. The asthenopia 
is readily explained by the illumination of the room, the glare of the 
light reflected from the cards, and the accommodative strain induced 
by demand for accurate vision at distances varying from one-third of a 
metre to one and one-half metres, and, in some cases, by the necessity 
for constant and vigorous mental action. 

Presbyopia is frequently a cause of headache. The crystalline lens 
has become hard and inelastic, its layers of fibres gliding on one another 
to an exceedingly limited degree while the innervation to the ciliary 
muscle remains practically unchanged. The contraction of the muscle 
in response to the innervation is also almost as strong as early in life. 

Ocular strain in the continuous effort to see clearly in the distance, 
such as the preacher in the pulpit and the actor on the stage, is a fre- 
quent cause of headache and other forms of asthenopia. The peculi- 
arity of this headache is its occurrence some hours after the strain is 
over. " Panorama" headache, or car-sickness, is less to be attributed 
to idiosyncrasy of individuals than the reflex of disordered ocular func- 

66 Trans.. Amer. Ophthal. Soc, 1901. 



820 THE EYE AND NERVOUS SYSTEM 

tion, brought about by the effort to fix distant and rapidly passing 
objects. The constant change of focus and line ( of direction of the 
visual axes and also the rapidly changing retinal image entail too much 
action of the accommodation, of the extraocular muscles, and of the 
retina and afferent visual impulses. 

Character. — The pain is sharp and neuralgic or dull and heavy, vary- 
ing according to the temperament of the individual, the degree of the 
error of refraction, the length of time during which the near use of 
the eyes is demanded, age, and the presence of muscular anomaly. The 
first exceeds in importance, as a cause of intensity of pain, all other 
causes, and demonstrates that the cause of the trouble is not only ocular 
anomaly, but the combination of an ocular anomaly with a susceptible 
impressionable disposition. Low defects in subjects enfeebled by dis- 
ease or whose power of resistance is weakened by suffering or mental 
distress may give rise to intense and constant headache, while in healthy 
and strong individuals equal defects, whether of refraction or muscular 
imbalance, are overcome or held in abeyance without any response what- 
ever of the nervous system. In H. and HAs. of between 1 and 2 D. 
the pain is apt to be constant during the working hours, and is aggra- 
vated by close work. In mixed astigmatism of moderate degree, the 
headache may be constant and associated with nausea and vomiting 
and not infrequently with symptoms of some nervous disorder. Near 
work is either impossible or accomplished only at the expense of intense 
suffering, and, if persisted in, results in chronic invalidism. The so- 
called neuralgia and migraine attributed by medical men and laymen 
to malaria, rheumatism, anaemia, and other general and vague diseases, 
is, in the majority of cases, only an expression of a disordered ocular 
function. For example : G. L. Walton, Boston Medical and Surgical 
Journal, has made an interesting study of migraine among individuals 
blind from infancy and others who later in life became partly blind. 

He found among 41 persons blind from infancy 80 per cent, had 
never had headache. 

Among 18 cases becoming completely blind in life only 5 were 
free from headache. 

Among 100 persons in good health only 31 per cent, had never suf- 
fered from headache. Seventeen had typical migraine (about one in 
six individuals), a marked contrast to the absence of typical migraine in 
the 41 cases of congenital and complete blindness. He believes that the 
explanation is to be sought in the frequency of eye-strain among those 
who have their sight, particularly among the partially blind, and its 
absence in the totally blind. 



LEUKOSES OCCASIONED BY EYE-STRAIX 821 

The neuralgia may be limited to one side of the face or may he 
bilateral. The supraorbital nerves are tender on pressure, or even 
painful spots located in the scalp simulating supraorbital neuritis may 
be present, and yet the cause be an ocular anomaly. 

The ocular headache assumes many phases. It cannot be certainly 
diagnosticated without an ocular examination and proper correction, 
but it is safe to predict in any given case that the combination of H. 
and HAs., or esophoria with an average nervous susceptibility, will be 
the cause of headache, which will be temporarily relieved by rest and 
permanently relieved by well adjusted correction, worn constantly. 

While headache is the most common form of asthenopia, other re- 
flexes not so directly referable to the use of the eyes are worthy of 
mention, — for instance, migraine, neurasthenia, insomnia, restlessness, 
nervousness, enuresis, urticaria, depression of spirits or mental apathy. 
Examples of each of these affections and others are found in medical 
literature. Their diagnosis and treatment is identical with that of 
ocular headache, indigestion, and other reflexes of eye-strain. 

Treatment. — The indications are twofold : first, the correction of 
the error of refraction by glasses and the muscular anomaly by appro- 
priate remedies ; second, hygienic and medical measures that will 
reduce the susceptibility of the nervous system, regulate the habits and 
modes of life, both as they pertain to the general health and more 
particularly to the ocular function. Ametropia may be corrected in 
individuals under 45 years of age who have hyperopic astigmatism 
only by learning the static refraction under paralysis of accommo- 
dation. The cycloplegic used is immaterial, provided it abolishes tem- 
porarily the contractility of the ciliary muscle. In myopes of more 
than 4 D. or 5 D. of myopia, cycloplegia is seldom necessary since such 
persons rarely suffer from accommodative headache and the ciliary 
muscle cannot conceal the optical defect. After the age of 45, cyclo- 
plegics are advisable only exceptionally, since the true refraction of the 
eye is manifest. 

The eyes are not exclusive factors in the production of headache, 
but no diagnosis is complete which ignores them.' 

SPASMODIC MOVEMENTS OF THE HEAD 
OTHER THAN CHOREIFORM. 

This affection may be due to organic disease of the cerebrum or 
of the meninges, to unnatural positions of the head in consequence of 
paralytic or functional imbalance of the ocular muscles, to traumatism 
of the neck or trunk, posture incident to some forms of chronic invalid- 



822 THE EYE AND KEKVOUS SYSTEM 

ism, rheumatism, and, possibly, to other deformities by which the 
physical equilibrium is disturbed. In the adult, head shaking is a 
symptom of paralysis agitans, disseminated sclerosis, general paralysis 
of the insane, aneurism and abscess of the brain. Head tilting is asso- 
ciated with functional and organic ocular muscle defects, oblique non- 
compensating bilateral astigmatism, traumatic or diseased contraction 
and paralysis of the neck muscles on one side, and to disease of the 
cerebrospinal system. In infants, involuntary rapid oscillation or other 
movements of the head, variously designated as head shaking, eclampsia 
nutans, head banging, salaam, convulsions of infancy, head bowing, 
and nodding convulsions, is accredited to arrested development of the 
nervous system, and is found in association with rickets, epilepsy, im- 
paired intellect, and physical deformities. " Sometimes head shaking 
in infants is a form of half-involuntary movements, twitchings, antics, 
and grimaces to which children are subject. In this case there is no 
fear of epilepsy, and recovery is the rule." 6T 

The position of the head assumed in compensation of heterophoria, 
heterotropia, and errors of refraction, is independent of central lesions 
and is purely voluntary. It is not in any sense a symptom of disease 
or a disease itself. It resembles the habit spasm or contractions of the 
facial muscles common in individuals in whom the visual act is accom- 
plished only by undue muscular strain. The direction of the tilting 
of the head characterizes to a certain extent the variety of defect. Thus, 
in paralysis of the externus of the right eye, the eye is deviated to the 
left and the face is rotated to the right in order to bring the visual 
line of the right eye parallel to that of the left. In paralysis of the 
motor oculi of the right eye the right cornea is deviated towards the 
temple and the face is turned to the left; in paralysis of the elevators 
of the cornea the chin is raised in order to bring the depressed axis 
of the paralyzed eye in line with that of the sound eye; in paralysis 
of the depressors the head is tilted forward with the chin receding. 
Bard 68 states that ocular deviation and rotation of the head after 
hemiplegia is due to active movements of the muscular groups of the 
unaffected side automatically governed by the unilateral action of cen- 
tral perceptions, or simply from the reflex power of the sensorimotor 
centres. Sight predominates, but other senses take part, particularly 
hearing and those of locality and equilibrium. But slightly influenced 
by the seat of the lesion, it is affected by the gravity, extent, sudden- 
ness and intensity of the shock and by the degree of coma. Both ocular 

67 Samuel Gee, St. Barthol. Hosp. Rep., 1886. 

68 Semaine Medicale, January 13, 1904. 



LEUKOSES OCCASIONED BY EYE-STRAIN 823 

deviation and head rotation disappear either on recovery of the affected 
hemisphere or extension of the lesion to the other side of the brain. 
In heterophoria — latent sqnint — tilting of the head or face is less'notice- 
able than in heterotropia (manifest squint), since in the former there 
is no actual deviation of the visual axes. The tension of the weaker 
set of muscles is partly substituted by contraction of the occipito- 
frontalis, corrugator supercilii, or the orbicularis palpebrarum. The 
undue stimulation of the facial muscles may be extended to include 
adjacent muscles of the neck or back, by the contraction of which the 
erect posture of the head or trunk is modified. Lewis 69 reports a case 
of pseudotorticollis in a girl eight years old. She complained of frequent 
headache during the winter school session. Previously a good scholar, 
she became dull, inattentive, irritable, and thin. During the summer 
vacation she gained in weight and the headaches were less, but improve- 
ment was cut short by resumption of school studies. The symptoms 
returned, aggravated by marginal blepharitis and lachrymation. She 
held her head in a depressed position downwards to the right side, so 
that the eyes when fixing an object directly in front were directed up- 
wards and to the left, the upper lids covering the irides almost down 
to the pupillary margins. Slow rhythmic swaying of the head when 
looking at the test letters was a constant symptom. There was no stiff- 
ness of the cervical muscles. The oblique position of the head was 
maintained in the endeavor to overcome the imperfect vision due to 
the need of mixed cylinders (R. — 75 = -\-2° ax. 100) right hyper- 
phoria of 3%° and esophoria 2°. In testing the near vision the same 
rhythmic swaying was noticed. Gould 70 relates an interesting and 
instructive case of torticollis and spinal curvature due to eye-strain 
(from astigmatism). He says the case should be interesting to the 
general physician, orthopedic surgeon, and oculist, because the patient 
was of tuberculous parentage, and it was feared that she would develop 
tuberculosis. The symptoms simulating tuberculosis were soon found 
to be caused by the false position of the head, that in turn by wry- 
neck which had caused spinal curvature. The patient, a girl of nine- 
teen, complained of severe and protracted headache, sore eyes, and 
pain in the eyeballs. The erect position was resumed and all the symp- 
toms both of eye-strain and tuberculosis disappeared after correction 
of compound hyperopic astigmatism. Without her glasses the head 
takes its former abnormal position, the right shoulder drops, the back 
becomes more humped, etc. The regained health is primarily due to 

69 Oph. Rec, 1903, No. 12. 
"American Medicine, March 25, 1904. 



824 THE EYE AND KERVOUS SYSTEM 

the glasses and secondarily to the proper position of the body, increased 
lung capacity, and better oxygenation of the blood. A second case of 
torticollis was a woman of 25, with high degree of astigmatism in 
both eyes, axes 170°, a defect which would not allow binocular vision 
with the head in its normal position. Gould alludes briefly to other 
cases, whose histories are confirmative of the causation of torticollis 
and spinal curvature by ocular defects and the cure of those affections 
by relief of the eye-strain. 

Wadsworth 71 reports a case of spastic torticollis in a boy of four- 
teen which had persisted since infancy. The boy was healthy and 
had good muscular development. He sat or stood with his head tipped 
to the right and inclined considerably forward, his right shoulder lower 
than the left and the spine laterally curved, with convexity to the left. 
The ocular excursions were good in all directions. In ordinary posi- 
tions, with fixations for near or distance, the left eye stood out about 
one degree higher than the right. Only when the eyes were turned 
strongly down and to the left, while at the same time the head was 
inclined forward and to the right, did he apparently get binocular fixa- 
tion. The superior rectus was divided, with the result that the body 
has been much straighter ever since, and is still improving. 

Walton 72 reports the case of a boy 12 years of age who had a 
general chorea, head jerking being the most marked symptom. The 
child had an esophoria of 6°. His internal recti were tenotomized, and, 
when seen two weeks after the operation, the twitchings had almost 
entirely ceased. 

Recurrence of head shaking, head tilting, and torticollis may be 
expected upon the return of the muscular insufficiencies. Should they 
relapse following a change in the refraction of the eyes, especially in 
the axis of the astigmatism, they may be again relieved by restoration 
of normal ocular conditions. 

Posey 73 classifies the head movements into (1) physiologic, or " the 
many and varied movements which the head makes through the medium 
of the neck muscles;" (2) abnormal independent, but simultaneous, 
or the accidentally simultaneous, occurrence of nystagmus and head 
tremor; (3) compensatory, or a vicarious position of the head assumed 
to compensate for some imperfect action of one or more of the extra- 
ocular muscles, and (4) related, but not compensatory, or those anoma- 

71 Trans. Amer. Ophthl. Soc, vol. v. 

72 Boston Medical and Surgical Journal, 1892. 

73 Associated Movements of the Head and Eyes, Journal of the American Medical 
Association, November 29, 1902. 



NEUROSES OCCASIONED BY EYE-STRAIN 825 

lous movements of both head and eyes, the result of central lesion. 
Under the third heading he refers to cases reported by Wadsworth, 
Risley, and Wilder, and adds one case of his own. Under the fourth 
class he describes a new case. 

Head Bowing of Infants. — This affection is essentially different 
in its pathology and etiology from the head movings or tiltings of adults, 
symptomatic or ocular disturbances. In infants the affection is attribu- 
table to organic changes in the brain or its membranes, the nature of 
which is unknown. Another and entirely distinct form of eye, head, 
and hand movements, which should not be confounded with the true 
eclampsia nutans, is that sometimes seen in children whose vision is 
reduced to the perception of light from ophthalmia neonatorum, or 
congenital cataract. In the latter affection, nystagmus is invariably 
present. The head is moved slowly, horizontally, and vertically and 
one or both hands are held between the eyes and the light and rapidly 
moved within a narrow area. In the former the infants are rhachitic, 
hydrocephalic, strumous, or tubercular. William Nestham, 74 who seems 
to have studied this affection with great thoroughness, says, " There 
is evidently in this malady a family alliance with epilepsy, and hence, 
as has been demonstrated by the pregoing cases, it often passes into 
epilepsy or other form of infantile convulsions. Tetanoid symptoms 
also sometimes occur during its progress." The disease has been at- 
tributed to dentition, irritation of spinal nerves from the presence of 
worms or other irritating matter in the body and also to atmospheric 
changes. His conception of the morbid action is that the essential char- 
acter of this malady is inflammatory action in the weak or strumous, 
which in all probability commences in the membranes investing the 
medulla oblongata. It is early extended to the membranes covering 
the base of the brain, and is succeeded by disease of the vessels supply- 
ing and nourishing the organ itself. This condition is then followed 
by exudations of lymph or serum, the locality of these effusions being 
determined by the constitution and local circumstances, and, as a con- 
sequence of these exudations, paralysis is produced from pressure. The 
regular nutrition of the brain is interrupted, and, in the more aggra- 
vated cases, the organ becomes so deteriorated as to lose all power of 
carrying on the intellectual functions. 

The rapidity and frequency of the head movements differ in differ- 
ent children, and in the same child at different times. The movements 
may recur at intervals of months, each spasm continuing a few min- 



London Medical Record, May 14, vol. xi. 1881. 



826 THE EYE AKD NEKVOUS SYSTEM 

utes, or they may be almost incessant for several hours. The head 
is slowly alloAved to fall backward, possibly because the child is too 
feeble to hold it erect, and, by a jerk, restored to its upright position, 
or it is thrown violently and rapidly from side to side, " banged" for- 
wards and backwards into an unresisting substance, as a pillow. The 
spasms may be associated with fainting or unconsciousness, or may 
occur during sleep or immediately upon awakening. Dr. Stephen Mac- 
kenzie 75 reported two cases, each presenting a series of more or less 
rhythmical movements of the head, partly consisting of a nodding of 
the head forwards and partly of a rotary movement. In each there 
was nystagmus, which in one case was almost entirely confined to one 
eye. The nystagmus was increased, or evoked if absent, by restraining 
the movements of the head. The nodding ceased during sleep. 

Thomson 76 says that more than three-fourths of the published cases 
of spasmas nutans, or head jerking, in infants begin between four and 
twelve months of age. This age corresponds to the period during which 
the child is slowly learning to co-ordinate the movements of the eyes 
with those of the head. Up to the fourth month of age the infant has 
great difficulty in moving the two sets of ocular muscles together, and 
even towards the end of the first year it has very little skill in following 
the object moving with his head and eyes. Cases have been described 
beginning as early as one month 77 and six weeks old, 78 but no details 
have been published of any commencing later than the twentieth month. 
In the case of second attacks, however, the symptoms may still be 
present in the third year. 

The prognosis is not necessarily unfavorable. When the affection 
is due to the reflex irritation of dentition, intestinal worms, phimosis, 
and other curable causes, complete recovery ensues upon removal of 
the irritation, provided the infant's health has not been too seriously 
undermined. 

Abt 79 saw a case of spasmus nutans with rotary nystagmus follow- 
ing injury to the head. Examination of the eyes showed that the pupils 
were regular and reacted normally. The media and fundi were healthy 
and there was no strabismus present. The head-nodding was almost 
continuous. Bromide of sodium, either alone or in combination with 
belladonna, was administered, and in six weeks the patient recovered. 

75 Lancet, vol. i. 1886. 

76 Scottish Medical and Surgical Journal, 1900. 

77 Schonberg, Norsk, Magaz. for Laegevidenskaben, 1891. 

78 Gee, St. Barthol. Hosp. Reports for 1886. 

79 Journal of the American Medical Association, February, 1900. 



LEUKOSES OCCASIONED BY EYE-STKAIN 827 

The infants who survive the disease of the cerebrospinal system of 
which the head nodding is a symptom, are usually permanently en- 
feebled, either physically or mentally. The ophthalmoscopic findings 
are, as a rule, negative, but should optic neuritis or optic nerve atrophy 
be a complication the prognosis is unfavorably affected. 

Treatment. — The principle upon which the treatment is based varies 
according to the most probable cause of the affection. Should spinal 
irritation be suspected, calomel to keep the secretions in order and 
tonics to support the strength are indicated. If dental irritation be 
present, thorough and repeated lancing of the gums is called for. The 
avoidance of everything that tends to increase cerebral action should 
be a part of the treatment. The bromides are useful. Nourishing 
diet at regular and frequent intervals, abundance of fresh air and sun- 
light, well ventilated sleeping-rooms, suitable clothing, and other 
hygienic measures, constitute an essential part of the treatment. 



CHAPTEE XIX. 

EXOPHTHALMIC GOITRE. 

By WILLIAM CAMPBELL POSEY, M.D. 

Exophthalmic goitre, Graves's disease, or, as it is designated in 
Europe, Basedow's disease, is an affection characterized in most cases 
by a group of symptoms which consist of increased frequency of the 
pulse, enlargement of the thyroid gland, the presence of certain charac- 
teristic ocular changes, a fine tremor, and nervous irritability. The 
disease is essentially chronic in its course and is of unknown origin, 
though probably dependent upon some disturbance of the central nervous 
system induced primarily by changes in the thyroid gland. 

Although others had practically described the disease, in reports of 
cases which were undoubtedly true instances of exophthalmic goitre, the 
honor of recognition of the symptoms as belonging to a distinct and 
separate morbid process should be accorded to Graves (1835), though 
it was not until five years later (1840) that Basedow, ignorant of 
Graves's discovery, fully described the disease with its cardinal symp- 
toms. 

SYMPTOMATOLOGY. 

All the Rve primary symptoms which have just been mentioned 
are not always present at the same time in every case, and one or more 
may be absent during the entire course of the disease; the tachycardia 
and the nervous irritability appear, however, to be present in all cases. 
In addition to these cardinal symptoms, there are other secondary symp- 
toms which are quite frequently present ; these include changes in the 
alimentary canal, in the respiratory and genito-urinary organs, and in 
the skin. 

Vascular System. — Increased Frequency of the Pulse; Tachycardia. — 
The most constant and usually the first symptom of exophthalmic goitre 
is a marked increase in the force and frequency of the contraction of 
the heart; the pulse may beat as rapidly as 120 to 160 times in the 
minute, and even 200 beats have been recorded; it rarely sinks to 
less than 100. The action of the heart is often irregular and may be 
at times intermittent; it is usually perceptible to the patient, espe- 
cially if it be even slightly increased by exertion or excitement. The 
828 



EXOPHTHALMIC GOITKE 829 

cardiac impulse is strong and extends over a larger area than is normal. 
In the early stages the sounds of the heart are usually unaffected, but 
later, as a consequence of the prolonged increased activity in the cardiac 
action, the sounds become louder than normal, and Gowers 1 is authority 
for the statement that they may be so intense at times as to be heard 
as far as four feet from the patient. 

Murmurs present in the early stages of the disease are usually of 
anaemic origin, but later actual murmurs from faulty valvular action, 
as a consequence of hypertrophy and dilatation of the heart, may arise. 
Pulsation in the arteries is common and can be readily seen and felt in 
the large arteries at the root of the neck and even in the peripheral 
vessels ; in thin subjects pulsation can be demonstrated in the abdominal 
aorta. 

Nervous System. — Tremor and Nervous Irritability. — Tremor is prob- 
ably present in all cases at some time or other; though usually an 
early symptom, it may not manifest itself until after other symp- 
toms have appeared. It is most marked in the extremities, par- 
ticularly the upper, though it may, however, extend over the entire 
body. The character of the tremor varies, but is usually fine and 
regular. In cases where the exophthalmos and goitre have been limited 
to one side of the body, the tremor may be unilateral also. It is best 
elicited by requesting the patient to stretch out the hands with the palms 
directed downward, when, if too slight to be seen, by laying the palm of 
the hand lightly upon that of the patient, the observer will note that 
the trembling is not in the fingers alone, but that the entire hand moves, 
this being occasioned by a spasmodic action of the flexor and extensor 
muscles of the wrist. The tremor is increased by excitement or by 
attempting to perform actions with extreme care, and resembles the 
tremor of fatigue and weakness in being a little more frequent in time 
than that of paralysis agitans or of disseminated sclerosis (page 90S). 

In nearly all cases of Graves's disease there is a slight mental change 
which usually manifests itself in an unwonted irritability and rest- 
lessness. There may be times when the patient regains his usual spirits, 
but he soon lapses again into a peevish and depressed state. In other 
cases the mental derangement takes the form of a nervous expectancy, 
and the patient is in a flutter of excitement about trifles. Xervous 
symptoms, as a rule, develop in individuals who are predisposed by a 
neurotic ancestry, but they may appear in subjects who were quiet and 
unexcitable or even phlegmatic before the disease manifests itself. 

In addition to this general nervousness, there are not infrequently 
other nervous symptoms which are of a functional rather than of an 



830 



THE EYE AND 1STEKVOUS SYSTEM 



organic nature. Hysteria is often present and may occur under various 
forms, though some variety of anaesthesia or paralysis is the most 
common. Many of the convulsions which occur in the course of exoph- 
thalmic goitre are of an hysterical origin. Headache and migraine 
in its typical form, neuralgia, dizziness, ringing in the ears, and other 
similar neuroses have also been met with. 

Actual insanity may supervene, taking the form of melancholia and 
mania or, more rarely, that of recurrent mania and general paralysis. 
The prognosis is always rendered grave by this complication, as death 
ensues in some cases. 

Paralyses of cranial nerves are not very unusual, probably from 
nuclear involvement. Symptoms of tabes have been observed in a 
number of cases ; paraplegia may manifest itself, either with flaccidity 
or rigidity of the legs, and Charcot 2 and Maude 3 have observed an 
inability to stand or walk, while the legs can be freely moved when 
lying down (astasia-abasia) , as an early symptom. 




Fig. 1.— A case of exophthalmic goitre. (From Wilbrand and Saenger.) 



Rarer complications include chorea, tetany, chronic muscular atro- 
phy, paralysis agitans, Thomsen's disease, rheumatoid arthritis, and 
osteomalacia. 

The Enlargement of the Thyroid Gland. — This usually occurs 
simultaneously with the exophthalmos. It may, however, exist for 
some years before any of the other symptoms of exophthalmic goitre 



EXOPHTHALMIC GOITRE 831 

appear. Of eighty-seven cases seen by Murray, in which either one 
or two symptoms appeared simultaneously before the others, an enlarge- 
ment of the thyroid gland was the first symptom of the disease in 
forty-three. Though not so constant a symptom as the tremor, it is 
present in most of the cases, Murray finding enlargement of the gland 
absent during some period of the disease in but three out of one hundred 
and twenty cases recorded by him (2% per cent.). Griffith 4 failed 
to find an enlargement at the time of examination in thirteen out of 
thirty cases. The gland, as a rule, is but moderately enlarged, and 
rarely attains the size of parenchymatous goitres. The enlargement 
is usually symmetrical upon both sides, though it frequently happens, 
especially when the exophthalmos is unilateral, that one lobe is more 
enlarged than the other; when this is the case the right lobe is usually 
the larger. 

The vessels of the growth are usually much dilated, and pulsation 
of the entire gland is generally palpable and visible as well, especially 
when the heart's action has been increased by excitement. A thrill can 
generally be felt on the gland, and the ear will detect a loud systolic 
murmur or often a bruit de diable. Guttman considers a double mur- 
mur to be pathognomonic. 

THE OCULAR SYMPTOMS. 

These constitute a very striking feature of the disease, and though 
none of them, when existing alone, can be considered to be pathog- 
nomonic of exophthalmic goitre, the association of one or more of the 
most significant with but one of the other cardinal symptoms of the 
disease is sufficient to establish the diagnosis of exophthalmic goitre. 
They may best be studied in the following order : 

The Exophthalmus. — Actual protrusion is not always easy to deter- 
mine, especially as the development of the exophthalmos is usually 
gradual, and the clinician will frequently be compelled to rely upon 
the observation of the patient or his family and friends or to have 
recourse to photographs taken earlier in life before it can be definitely 
asserted that an actual protrusion of the globe, has occurred. Care 
must also be taken to differentiate unusual prominence of the eyeball 
due to increased length in the axis of the globe (myopia or anterior 
staphyloma) or to shallow orbits, from true exophthalmos. The degree 
of the exophthalmus varies greatly in different cases, in some being so 
slight that it occasions a mere staring expression, while in others it is so 
pronounced that the lids cannot be completely closed over the globe; 
in extreme cases the eyeball may even be dislocated in advance of the 
lids (Pain.). 5 



832 



THE EYE AND NERVOUS SYSTEM 



The degree of the exophthalmus may change from day to day, the 
protrusion being made greater by whatever increases the heart's action. 
Its progress also is not always uniform, for there are frequent periods 
when the globe appears to remain stationary. In some cases the exoph- 
thalmus has appeared only under special circumstances ; thus De Mussy 
found it in a case only during choreic attacks and Savage in another 
case at the time of menstruation alone, when there were symptoms of 
mental disturbance. 

The eyeballs can be pushed easily back into the orbits, though they 
come rapidly forward as soon as the pressure is relieved. The protru- 
sion is generally directly forwards, and both eyes are, as a rule, simi- 
larly affected, though the exophthalmos may be limited to one eye. 

A case of this nature was presented by Dr. Swindells and the writer 
before the College of Physicians of Philadelphia. In this patient, a 
female, aged 22, the left eye became suddenly proptosed two years pre- 
viously following a fright. She had been nervous for some years and 




w -•• 




Fig. 2.— Unilateral exophthalmus. 



had suffered from irregular menstruation. Her mother was neurotic 
and had goitre, but no exophthalmus. A maternal grandmother had 
died of cancer of the throat. The exophthalmus in the left eye was of 
moderate degree and the right eye was normal. All lid signs were 
absent except infrequent winking. The gland was bilaterally enlarged, 
though the right side was the larger. Marked improvement in the gen- 
eral and local condition followed a strict rest cure, galvanism, and syrup 
of hydriodic acid. 

A comprehensive monograph upon the subject of unilateral exoph- 
thalmus was contributed by Fridenburg in 1895. This author reported 
one case in his own practice and one from Jacoby's, and gave 6 in addi- 
tion the notes of ten additional cases which he had collected from the 
literature. Since the publication of this article additional cases have 



EXOPHTHALMIC GOITRE 



833 



been reported by Sichels, 7 Volkels, 8 Prael, 9 Mauthner, 10 Mooren, 11 
Bosner, 12 Emmert, 13 De Giovanni, 14 Barella, 15 Hitscham, 16 Mendel, 17 
Trousseau, 18 and Bistis. 19 

A number of observers have remarked that in many of these cases 
the enlargement of the thyroid was limited to one side of the gland, and 
that upon the side on which the eye was proptosed. The majority of 
the cases, however, show no such association, and no special significance 
can be attached to the occurrence. Sattler 20 explains unilateral exoph- 
thalmus by a disturbance of the vasomotor centres of the orbit of the 
affected side. 

The Various Lid Signs. — The Graefe Sign. — This sign, which con- 
sists in the impairment of the consensual downward movement of the 
upper lid with that of the globe (see page 351), was described by Von 
Graefe 21 in 1864. Though present in many cases of exophthalmic 
goitre, it cannot be considered to be pathognomonic of this disease, for 
it may not infrequently be demonstrated upon the eyes of healthy indi- 




Fig. 3.— Graefe's lid sign in exophthalmic goitre. (From Wilbrand and Saenger.; 



viduals. Thus, Sharkey 22 observed it twelve times among six hundred 

and thirteen cases of all kinds of disease, exophthalmic goitre excluded. 

The same author found, too, that a large proportion of healthy people 

can voluntarily produce this sign and the one next to be described — the 

Dalrymple — by staring at an object in front of them. The writer has 

frequently noticed that when patients are first made to fix a near object 

in testing the balance of the extraocular muscles, the palpebral fissure 
53 



834 



THE EYE AND NERVOUS SYSTEM 



frequently widens and the Graefe sign can be demonstrated. In such 
cases, however, in a few moments, especially after several trials have 
been made, the fissures assume their normal width, and the normal 
relationship between the movement of the lids and the globe is regained, 
showing, in the opinion of the writer, that the anomalous behavior of 
the lids and globe was due to a temporary incoordination produced by 
a confusion in the mind of the patient as to what particular demand 
was being made upon him. ITughlings Jackson, 23 Wilbrand and Saen- 
ger, 24 and Elatan 25 also have observed the symptom in healthy indi- 
viduals. Raymond 26 noted it in two cases of Thomsen's disease. 
Kocher 27 and Jessup have shown that similar phenomena, but to a 
lesser degree, may be produced by instilling cocaine into the conjunctiva, 
and deemed the sign to be occasioned in these cases by a direct stimu- 
lation of the sympathetic nerve-enclings, and a consequent contraction 
of the orbital muscles supplied by these nerves. As further evidence 
that the Graefe sign is not pathognomonic of exophthalmic goitre, it 




Fig. 4.— Dalrymple lid sign in a healthy subject. 

may be remarked that there are many cases of this disease in which 
it does not occur at all ; thus, Mannheim 28 observed it but eighteen 
times in forty-one cases, and Russell Reynolds 29 in but four out of 
forty-nine. Passler 30 saw it but nine times among fifty-eight cases ; 
Bruns 31 ten times out of forty-two cases; Platan 25 sixteen times 
among thirty cases, and Murray 32 thirty-six times among ninety-one 
cases. 

An impaired movement similar to that observed in the upper lids has 



EXOPHTHALMIC GOITEE 835 

been noted upon several occasions in the ascent of the lower lids also, but 
as Maude has pointed out, this is never so marked as in the upper lid, on 
account of the greater range in the downward rotation of the globes. 
The Graefe sign occurs quite independently of the degree of the exoph- 
thalmus, and may disappear during the course of the disease and re- 
appear again without the exophthalmus having changed its degree. 

The Dalrymple Sign.* — This refers to the peculiar staring expression 
which patients with exophthalmic goitre present. It is occasioned by 
an abnormal widening of the palpebral fissure, due to retraction of the 
eyelids, and gives rise to one of the most striking symptoms of the 
disease (Fig. 4). (Compare Page 350). 

The Stellwag Sign. — This is the name applied to the infrequency and 
incompleteness with which the act of winking is performed. It is a 
very marked symptom in some cases and may lead to corneal ulceration. 

Too frequent nictitation has been observed in some cases; thus, 
Hitschmann 33 has reported a case where the act of winking was per- 
formed about thirty times a minute. 

Both the Dalrymple and Stellwag signs may be confined to one eye, 
though they usually occur in both. They are more marked at times 
than at others, being increased by fatigue, excitement, or anxiety. They 
may each exist independently or in combination with one another or 
with exophthalmos and the Graefe sign. The figures of Wilbrand and 
Saenger 34 are of interest in this connection. Among thirty-nine cases 
of exophthalmic goitre seen by these authors, exophthalmus was present 
in twenty-seven cases, in twenty-three of which the exophthalmus was 
bilateral and in four unilateral. Among these twenty-three cases, ex- 
ophthalmus, Graefe sign and Stellwag sign occurred simultaneously in 
five cases ; exophthalmus, Graefe sign, and Stellwag sign occurred simul- 
taneously in four cases in the same eye; exophthalmus, Graefe sign, 
and Stellwag sign in fourteen cases; exophthalmus, Graefe sign, and 
Stellwag sign in four cases; exopthalmus, Graefe sign, and Stellwag 
sign were absent in six cases; Graefe and Stellwag signs in one case; 
Stellwag sign alone in two cases ; Graefe sign only in three cases. 

Mannheim's 35 statistics show that among forty-one cases of exoph- 
thalmic goitre the Graefe, Dalrymple, and Stellwag signs existed at the 
same time in ten cases, and that six showed the Graefe sign without 
the other two, while the Graefe sign was absent and the two others 
present in four cases. 

* This sign is frequently included as part of the Stellwag sign, but the credit 
of its discovery should be given to Dalrymple, who first pointed it out twenty years 
before. 



836 THE EYE AND NERVOUS SYSTEM 

Despite these figures, however, it is probable that if carefully 
searched for, all three of the signs will be found at some stage of the 
disease, and while their presence cannot be considered to be pathog- 
nomonic of exophthalmic goitre, it is of great value in confirming other 
symptoms. 

The Mbbius Sign. — According to Mobius, 36 unilateral convergence 
is normal in exophthalmic goitre, but the force of binocular convergence 
is sensibly diminished, quite independent of any mechanical obstruction 
offered to the proper convergence of the globes by extreme exophthalmos 
or from any paralysis of the ocular muscles. This weakness does not 
occasion diplopia, but evidences itself in a troublesome asthenopia, which 
frequently leads the patient to consult an oculist for relief. 

Paralysis of Extraocular Muscles. — In cases of marked exophthal- 
mos there is frequently more or less restriction in the movements of 
excursion of the eyeballs, although, as the degree of this limitation is 
about the same in both eyes, the patient suffers but little upon that 
account. There may, however, be actual palsy of the extraocular 
muscles. 

Interesting monographs upon this subject have been written by 
Ballet, 37 Liebrecht, 38 Buschan, 39 Mannheim, 40 and Mobius. 41 It would 
appear, from the cases reported and cited by these authors, that the 
paralysis may affect a single muscle or group of muscles of one eye, 
or even all the extrinsic muscles of one eye ; and that occasionally one 
or more muscles of both eyes are simultaneously affected. Palsy of the 
ciliary muscle or iris is not recorded, nor of any of the associated move- 
ments of the eyes, with the exception of reports by Schmidt-Bimpler 42 
and Vossius 43 of cases of paralysis of convergence. Palsy of the levator 
palpebral superioris in conjunction with palsy of the other branches of 
the third nerve is rare, but is rather more common without such asso- 
ciation. The palsy may come on early in the course of the disease, but 
in most cases it is a late manifestation; though usually persistent, it 
may be but a transient condition, the muscles regaining their full 
strength. 

The palsy may occur without paralysis of other cranial nerves, or 
it may be associated with a similar affection of one or more of them; 
thus, Bristowe 44 has recorded the case of a young man who developed 
the symptoms of ophthalmoplegia externa three years after the ordinary 
symptoms of Graves's disease had manifested themselves. There was 
also right hemianesthesia, with color-blindness and loss of taste and 
smell on the same side, some palpitation and dyspnoea with headache 
and sickness. After a period of some months, epileptic fits came on, 



EXOPHTHALMIC GOITKE 837 

and later on hemorrhages from both ears. Death finally occurred from 
bronchitis. Autopsy was negative. 

Similarly, in a case reported by Warner 45 there was binocular exter- 
nal ophthalmoplegia with palsy of the facial and trigeminal nerves. 
Jendrassik 46 saw paresis of the muscles of mastication, in addition to 
palsy of the rotary muscles of both globes, as well as most of the mus- 
cles of the face and palate. Of interest in this connection also are four 
cases reported by Ballet; 3T the first, the subject of severe hysteria, in 
addition to ophthalmoplegia externa, presented a number of bulbar 
symptoms, — i.e., palsy of both facials and paresis of the hypoglossus. 
In the second, also the subject of severe hysteria, there was right-sided 
hemiana?sthesia and loss of smell and taste on the same side. Autopsy 
showed no visible change in the central nervous system. In a third 
case, in addition to palsy of both external rectus muscles, there was 
right-sided hemiplegia and hemianesthesia, with epileptic attacks. The 
fourth case was an instance of ophthalmoplegia externa with paresis 
of the facial and hypoglossus. Chevalier 47 saw palsy of the externi 
and oblique muscles associated with disturbance in the supply of the 
fifth and seventh nerves and of speech. 

In a case of Maude's 48 the lesion seemed to spread over the entire 
region of the nuclei of the extraocular muscles and those neighboring 
upon them ; paresis of the external rectus, and probably of the superior 
oblique, being preceded by palsy of the facial, and quickly followed by 
general ophthalmoplegia. 

Stellwag's 49 case was one of paralysis of the lateral rotators (the 
ocular axes being parallel and convergence undisturbed), and later palsy 
of both externi with convergent strabismus and diplopia developed with 
transient anaesthesia and restricted movement of the left half of the 
upper legs. 

In a case reported by Fereol, 50 of a man 41 years old, six months 
after the appearance of the initial symptoms of exophthalmic goitre 
there were headache, vomiting and dizziness, tremor, with disturbed 
gait, and a disposition to face to the right ; then diplopia, the result of 
right-sided trochlearis palsy. There was also right-sided diminution 
of motion and hyperalgesia, while on the left side there was a diminu- 
tion in sensation of pain. 

Quite recently Voss, 51 of St. Petersburg, has reported two additional 
cases of palsy of the extraocular muscles in exophthalmic goitre. The 
palsy in the first case had affected the right superior and external rectus 
muscles of the right eye. and the inferior and external rectus muscles 
of the left eye. There Avas also a partial palsy of the right facial nerve, 
with some cerebral symptoms. 



838 



THE EYE AND NEKVOUS SYSTEM 



The second case exhibited multiple lesions in the muscles of both 
eyes, the superior rectus muscle of the right eye being particularly 
affected. 

Voss refers to a case of Bartholow's where there was ptosis in asso- 
ciation with gangrene and neuritis of the lower extremities. 

Dyson 52 reported the case of a man with the symptoms of incipient 
Graves's disease, in which, in addition to slight exophthalmos and the 
Graefe lid sign, there was slight internal convergent strabismus and dila- 
tation of the pupil of the left eye. The patient was a chronic alcoholic. 

Bruns 53 saw ptosis on the left side, with palsy of the superior recti 
in both eyes, with the cardinal symptoms of exophthalmic goitre, but 
as the patient was the subject of a chronic nephritis, it was thought 
that the nuclear lesion might be referable to this source and not to 
Graves's disease. 

Wilbrand and Saenger 54 report a case with slight ptosis on the left 
side, with retraction of the upper lid, with Graefe lid sign, upon the 
right side. This was explained by a bilateral affection of the nuclei of 




Fig. 5.— Paralysis of eye muscles in exophthalmic goitre. 



the levator, — i.e., an irritation of the levator on the right and a palsy 
on the left side. 

De Giovanni 55 has reported a case of unilateral exophthalmos as a 
consequence of palsy of the extraocular muscles, especially of the rectus 
superior, in which goitre and other classical symptoms of Graves's dis- 
ease were present. 

The accompanying figure is that of a patient presented by the writer 
before the College of Physicians of Philadelphia, in whom there were 



EXOPHTHALMIC GOITKE 839 

all the cardinal signs of exophthalmic goitre, and, in addition, paralysis 
of all the extraocular muscles of the left eye, with the exception of the 
superior oblique. 

In addition to these cases of more or less complete ophthalmoplegia 
externa, others, though more uncommon, of palsy of a single eye-muscle 
have been reported. These have been grouped by Buschan, 56 as follows: 

(1) Cases in which diplopia was complained of, but the muscles at 
fault not ascertained: Trousseau Smith, Chvostek, Stellwag, Impac- 
cianti, Striimpell, F. Miiller, Lang-Pringle. (2) Cases of palsy of the 
rectus internus: West, Homen, Cohen, Westedt, and Romberg (rectus 
interni of both eyes) ; Schoch and Rothmann (rectus internus and ex- 
ternus). (3) Cases of palsy of the rectus externus: Kurella, Mac- 
kenzie (several cases), Eulenburg, Stellwag, Makeig Jones, Liebrecht, 
Dyson, Ballet, Cohen. (4) Cases of palsy of the rectus superior: Sol-" 
lier, Fischer-Degranges, Kahler, Roth, Schlesinger, Chvostek. (5) 
Cases of palsy of the trochlearis: Fereol. (6) Cases of palsy of the 
levator palpebras of both eyes: Maude, West. 

Changes in the Cornea. — Notwithstanding the exposure to which 
the eyeball is subjected by the widening of the palpebral fissure and 
by the extreme degree of exophthalmos in many instances, and to the 
lessening in its moisture by the infrequent winking, and, furthermore, 
by the areas of anaesthesia, which may be demonstrated in the cornea 
in some cases of exophthalmic goitre, ulceration of that membrane 
occurs but rarely, appearing to depend more upon individual predis- 
position than upon any of the foregoing factors, those who are feeble and 
cachectic as well as those in advanced years, being particularly liable 
to severe corneal ulceration. Corneal complications show a tendency 
to occur more frequently among men than women, which is the more 
striking, in view of the fact that exophthalmic goitre is far more com- 
mon among females. This may possibly be explained by the greater 
severity with which the disease attacks men and also by the advanced 
age at which they are affected. 

In cases where the cornea is but slightly diseased, its epithelium 
becomes thickened, giving a dull and lustreless appearance to that mem- 
brane. The conjunctival vessels are usually dilated, as a consequence 
of which the patient experiences sensations of more or less irritation 
and dryness of the eyes, especially after reading. In more severe cases 
one or more small yellowish-gray areas of infiltration appear under 
the epithelium. The epithelium over these areas is thrown into folds 
and finally breaks down into shallow ulcers. In other cases, where the 
exophthalmos is of extreme degree and the lids do not close and properly 



840 THE EYE AND NEKVOUS SYSTEM 

protect the cornea at night, the lower third of the cornea becomes cov- 
ered with thick yellow crusts, beneath which ulcers form, and not infre- 
quently necrosis of the cornea and perforation of the eyeball ensues. 
It should be remarked, however, that extensive ulceration may occur 
in individuals where there is but slight exophthalmos, and in cases even 
where there is a plentiful supply of tears, the process in such instances 
being similar to that observed in keratitis of a neuroparalytic origin, 
the so-called keratitis xerotica. 

Anomalies of Lachrymation. — Berger 57 called attention to a num- 
ber of cases in which he claims that epiphora was the initial symptom 
of exophthalmic goitre, and Vernean and Diaman Herger have reported 
similar cases. The first author regards the epiphora as a secretory 
neurosis of the lachrymal gland, being due to a reflex condition of sen- 
'sory excitation of the sympathetic vasomotor fibres. Most authors, 
however, agree with Sattler in ascribing the epiphora to two factors: 
(1) The increased width of the palpebral fissure, whereby a larger 
portion of the conjunctiva is exposed to the action of the air than is 
usual, and thus, being irritated, excites reflexly an increased flow of 
tears, and (2) the interference with the proper sucking up of the tears 
by the lachrymal apparatus, as a consequence of the imperfect and 
infrequent winking and closing of the lids. 

In cases where the disease is of long standing, there is frequently 
a diminution in the amount of tears, and an actual dryness of the eye 
ensues ; this may be accredited to a lessening in the sensibility of the 
sensory corneal and conjunctival nerves, as the result of exposure and 
irritation, and to a corresponding lessening in the reflex secretion of 
tears. (Compare Page 344.) 

Unusual Ocular Manifestations. — Obliteration of the Sulcus Orbito- 
palpebralis Superior. — In spite of the emaciation which is often seen in 
subjects of exophthalmos, the sulcus, which is usually deep under such 
circumstances, may be obliterated, and in its place a sac-like fulness of 
the lid may appear, w T hich is occasioned by the increase in the contents 
of the orbit due to the fulness and stretching of the blood-vessels. 

(Edema of the Lids. — (Edema of the lids is sometimes present ; it 
may be an early symptom, or may not come on until after some years. 
Such swelling is often very persistent, and may remain after all the 
other symptoms have disappeared. Mackenzie 58 considered it to be 
somewhat akin to the swelling of myxcedema and to be quite independent 
of the exophthalmos. 

A bag-like swelling of the under lid, with dilatation of the veins 
at the ciliary border, associated with severe pain in the forehead and 



EXOPHTHALMIC GOITRE 841 

over the vertex, has been described by Stellwag. 59 Vigouroux 60 found 
a false oedema of the lids to be of frequent occurrence. He considered 
this to be dependent upon paresis of the orbicularis, as he observed that 
when this muscle was made to contract by electric excitation, the swell- 
ing disappeared. 

Tremor of the Lids. — Homen 61 has noticed a tremor in the upper 
lids when the eyes were closed, which diifered from the tremor seen in 
the lids in other diseases of the nervous system and occasionally in 
health by the rapidity with which it manifested itself after the lids 
were closed, by its regularity and rhythm, and its extremely fine char- 
acter. A similar tremor was noted by Liebrecht. 62 In this case the 
motion in the lid could be stopped after a few moments by pressure 
upon the supraorbital and facial nerves. 

Nystagmus. — Cases of nystagmus have been reported by Bramwell 63 
and Freund. 64 The latter author believes the movements in the eye- 
balls, although differing in rhythm, to be analogous to the tremor in 
the extremities. 

Changes in the Fundus Oculi. — With the exception of a tortuosity and 
dilatation of the retinal veins which are present in some cases, changes 
in the retinal vessels are very uncommon. O. Becker 65 saw a dilata- 
tion of their walls and the presence of a pulse isochronous with the 
radial pulse in the retinal arteries in ten out of thirteen cases, but most 
observers have failed to substantiate these findings. 

In like manner,, changes in the optic nerves are uncommon, and the 
cases of double optic neuritis which have been recorded by Story 6G 
and Rieger and Eorster 67 and the two cases of optic atrophy seen by 
Emmet 68 should be regarded as complications rather than as symptoms 
of the disease. 

Refraction. — In thirty cases examined by him, Griffith 69 found the 
refraction to be normal in twenty-two instances. Of the remaining 
eight, four were myopic, one highly hypermetropic, and three astig- 
matic. 

Field of Vision. — Xotwithstanding the observations of Kast and Wil- 
brand, 70 who found concentric narrowing of the field of vision with or 
without impairment of the central vision or the color sense in subjects 
free from hysteria, but exhibiting more or less signs of cardiac palpi- 
tation, the visual fields in exophthalmic goitre, except in so far as they 
may be influenced by an accompanying hysterical condition, are normal 
(see page 638). 

The Cutaneous System.— The changes in the integument may affect 
(a) the vasomotor system, occasioning at times the so-called " tache cere- 



842 THE EYE AND NERVOUS SYSTEM 

bral," oedema, and peripheral changes in temperature. Of these the 
" tache cerebral/' the transient redness which follows even the slightest 
touch of the skin, is a not infrequent symptom of exophthalmic goitre, 
and was attributed by Trousseau 71 to a marked asthenia of the vaso- 
motor apparatus. (Edema may be either general or local. It occurs 
for the most part in debilitated subjects and is influenced by gravity, 
though there are other forms of circumscribed oedema probably akin 
to those observed in myxoedema. Increase in the bodily temperature 
has been noted not rarely, and may occur in all the stages of the disease 
without apparent cause. This actual increase in temperature is to be 
differentiated from the sensation of excessive heat, without increase in 
temperature, which is at times complained of and to which Charcot 
has assigned the name of thermophobia. 

(b) The glands, — excessive sweating. Excessive sweating upon 
slight cause is one of the most frequent symptoms of the disease, and 
may be local, as, for example, when it is confined to the hands, or, what 
is more often the case, it may involve the entire surface of the body. 
It may be confined to one-half of the body. Diarrhoea is sometimes most 
troublesome, and is thought to be a kind of intestinal sweating. 

(c) The skin — pigmentation. Pigmentation of the skin has been 
observed by a number of authors. In most cases it assumes a bronze 
tint, comparable to that in Addison's disease, though never so pro- 
nounced as in advanced cases of this affection. Vitiligo and urticaria 
eruption, and a double form of rash consisting of dusky papules like 
the eruption of measles in its later stages, have also been observed. An 
abnormal dryness of the skin has been noted in severe cases. 

It was noted by Vigouroux 72 that in many cases of exophthalmic 
goitre there was a decided decrease in the normal resistance offered by 
the skin to the passage of the galvanic current. This he thought was 
due to hyperidrosis. As this symptom does not occur in other diseases 
where the perspiration is abnormal, and may even occur in health, it 
cannot be considered to be pathognomonic. 

The hair. — Loss of hair frequently occurs from the scalp, axillae, 
and pubes. Thus, Mannheim 73 observed this symptom in fifteen out 
of forty-one cases, and Yeo 74 reports an interesting case where the 
hair fell out of the eyebrows and eyelids coincidently with the protru- 
sion of the eyeballs. It is of interest to note that the loss of hair is 
often unilateral. 

Respiratory System. — Disorders of respiration are not uncommon 
and are occasioned either by irritation or pressure. Among the former 
may be included the attacks of coughing which resemble laryngeal crises. 



EXOPHTHALMIC GOITEE 843 

In many of these cases the dyspnoea may be very great and death from 
asphyxia may ensne. There may be but little secretion, but at times 
there is a profuse bronchorea, which is conceded to be due to vasomotor 
paralysis. 

Digestive System. — Disturbance in the gastro-intestinal tract occurs 
in at least half of the cases, and usually takes the form of a serous diar- 
rhoea, which appears in paroxysms and without colicky pains. These 
attacks may be most persistent, and may even end fatally. 

Generative System. — Although many observers are of the opinion 
that menstrual disorders are a frequent cause of exophthalmic goitre, 
there is no evidence to prove this or to indicate that the quite frequent 
disturbances in menstruation which occur in conjunction with this dis- 
ease are more than functional derangement, the result of anaemia and 
chlorosis. 

Urinary System. — Polyuria is of common occurrence, and is de- 
pendent upon vasomotor activity, being analogous to the excessive sweat- 
ing and the diarrhoea. Transitory albuminuria has been reported in a 
number of cases. Glycosuria is a frequent symptom, depending in 
some cases upon true diabetes, in others upon simple so-called alimen- 
tary glycosuria. 

Anaemia, chlorosis, and any condition which lowers the vitality, such 
as prolonged illness, loss of blood, amenorrheea, sexual excess, the abuse 
of alcohol, and syphilis, may predispose to the development of the dis- 
ease ; cases have been reported also where diseases of the nose and pelvic 
organs were thought to have originated the symptoms. Among the 
exciting causes are sudden fright, great grief, prolonged fatigue, and 
violent physical strain. 

COURSE, PROGNOSIS, AND FREQUENCY. 

The disease begins, as a rule, very suddenly, symptoms of palpita- 
tion and tachycardia rapidly developing after a physical shock. These 
either persist, or, what is more common, subside, to be re-excited again 
by similar causes until the symptoms remain permanent. The future 
course of the disease is usually a Chronic one, and it may persist for 
years with occasional variations in severity. Complete recovery occurs 
in some cases, but, as a rule, some of the symptoms remain until death 
ensues from some intercurrent disease. 

Prognosis. — The prognosis, as far as life is concerned and also for 
a comparatively short duration of the disease, is good, provided the 
cases are submitted early in their course for treatment and that the 
subjects are young. The prognosis is more favorable in women than 



844 THE EYE AND NERVOUS SYSTEM 

in men. Though rapid onset may be followed by rapid recovery, re- 
covery is usually more rapid and permanent in cases where the symp- 
toms develop more slowly. The appearance of grave complications, 
such as vomiting, diarrhoea, and the signs of cardiac failure, are always 
of serious import, as they may occasion a fatal termination. Exoph- 
thalmic goitre of itself is seldom fatal. 

Frequency. — Exophthalmic goitre is a rare disease ; it occurs in 
all lands and shows a preference to affect individuals between 20 and 
30 years of age. Thus, of four hundred and ninety-five cases collected 
by Buschan (four hundred and seven females and eighty-eight males), 
the statistics were as follows : 

Females. Males. 

From two and a half to ten years of age 12 3 

From eleven to fifteen years of age 17 11 

From sixteen to twenty years of age 83 10 

From twenty-one to thirty years of age 144 19 

From thirty-one to forty years of age 79 17 

From forty-one to fifty years of age 51 18 

From fifty-one to sixty years of age 21 10 

Sex. — Buschan collected nine hundred and eighty cases in the litera- 
ture, and found of this number eight hundred and five to be women 
and one hundred and seventy-five men = 1 : 4, 6. 

PATHOLOGY. 

Since the recognition of Graves's disease as a distinct morbid entity 
numerous theories have been advanced to account for its causation, and 
it has variously been imputed to a dyscrasia, to intestinal intoxication, to 
disease of the nervous system, and, finally, to a disturbance in the action 
of the thyroid gland itself. Of these theories the most tenable are those 
which are based upon either a disease of the nervous system or of the 
thyroid gland. Regarding the former, it may be said that although 
observers have designated nearly every portion of the nervous system 
as the primary seat of the disease, our present knowledge of the subject 
permits us to eliminate all parts other than the medulla oblongata and 
the sympathetic nervous system. 

While it may be stated that it has been demonstrated that symptoms 
of exophthalmic goitre may be occasioned by interference with the nerve- 
centres in the medulla oblongata, and though definite lesions have been 
found in this part of the brain in some autopsies wdiich have been made 
upon fatal cases of this disease, there is not yet sufficient evidence to 
prove that an organic lesion of the medulla is necessary to produce 
exophthalmic goitre. JSTor is there ground to warrant the assertion that 
the sympathetic nervous system is primarily at fault, though many of 



EXOPHTHALMIC GOITKE 845 

the chief symptoms of the disease may be explained by an affection of 
that part of the nervous system, and though lesions have been found 
there in cases of Graves's disease. 

As regards the gland itself, while it is evident from microscopical 
studies that there is a great increase in the amount of the secreting gland 
tissue in exophthalmic goitre, and from other observations that it is not 
improbable that there is an alteration in the character of the secretion 
as well as its quantity, and that the symptoms of the disease may be 
due to an action of an excess of this altered secretion of the gland upon 
the tissues, and especially upon the nerve nuclei of the medulla, the 
entire question of the essential factor or factors in the production of 
exophthalmic goitre must still remain sub judice. 

TREATMENT. 

The treatment of Graves's disease resolves itself into a general 
hygienic, medical, electrical, and surgical. 

General Hygiene. — The chief indication in the treatment of Graves's 
disease is rest. Most patients do best upon a prolonged course of strictly 
enforced rest cure, the treatment being protracted over a period of one 
or more months of absolute rest in bed. Where this is impracticable 
or seems undesirable, bodily and mental rest, as far as is possible, should 
be installed, with the avoidance of all but the slightest amount of physi- 
cal exercise and the withdrawal of the patient from all contact with 
that which might worry or excite the mind. No specific regimen can 
be prescribed as suitable for all cases, the physician being compelled 
to adjust the amount of exercise and rest for each individual. All 
violent exercise should be proscribed, and gymnastic exercises, except 
perhaps the passive mechanical exercise employed by Bryson, are to be 
avoided. Patients do well by the sea, and a bracing air is usually of 
advantage. High altitudes should be avoided. Long sea-voyages are 
often beneficial, provided the patient is not subject to sea-sickness. 
Alcoholic stimulants are only to be prescribed when the heart is very 
w T eak or assimilation very poor. Tea, coffee, and tobacco should be pro- 
hibited. The free consumption of milk should be encouraged, and 
simple and readily digested foods selected ; but the diet should always 
be prescribed with reference to the conditions revealed after an analysis 
has been made of the gastric contents. If massage be administered, ir 
should be given gently and not too frequently. Cold baths are harmful ; 
warm baths and occasional shower-baths at 100° P. are of benefit. The 
application of Leiter's ice-coil over the thyroid gland and occasionally 
over the heart will often diminish the exophthalmos, as well as the 
vascular excitement. 



846 THE EYE AKD NEKVOUS SYSTEM 

Medicinal Treatment. — The indications to be met in the adminis- 
tration of drugs are to lessen the excessive and abnormal secretion of 
the thyroid gland, to quiet the general nervousness, to build up the 
general health and overcome anaemia. A most efficient means to reduce 
the size of the gland consists in rubbing a small piece of red iodide of 
mercury ointment over the gland each night until the skin becomes too 
tender for the patient to bear its further application. After a few days 
of interruption, this treatment may be resumed and continued with 
similar interruptions for several weeks or months, or the skin over the 
gland may be painted with tincture of iodine or anointed with a com- 
pound consisting of half a drachm of extract of belladonna and one 
ounce of iodine ointment. 

Belladonna may also be administered internally, beginning with ten 
minims of the tincture three times daily and continued until secretion 
has been appreciably affected. Iodine and the iodides have also been 
of advantage when given by the mouth in some cases, though in others 
they have been harmful. Bromides are of service in controlling the 
nervous irritability, and sulphonal, in addition to its action as an 
hypnotic, has been found to have a favorable effect upon the cardiac 
and general nervous systems. The valerianates, sumbul, and asafoetida 
are also of service. Musser relies as standards upon iron, cod-liver oil, 
and opium. Powdered opium is administered in doses of a quarter to 
a half of a grain to quiet the erythresium, control the diarrhoea, and 
stimulate the heart. Strophanthus is often of service, but contrary to 
what might be expected, drugs that slow the pulse, like digitalis and 
strophanthus, have but little influence in controlling the disease, though 
they should be administered when the heart is weak. Ergot is often 
of decided value, and iron and quinine are useful if there is ansemia. 

An extract of the thymus gland has done good in some cases, but 
the results from its use have been disappointing. Thyroid extract should 
always be used with caution, on account of its liability to increase the 
symptoms. Powdered oxide of zinc may be employed to check the 
sweating and control the diarrhoea. 

Electrical Treatment. — Galvanism is of decided value in many 
cases, and is best applied by placing the cathode over the angle of the 
jaw, first upon one side and then on the other, while the anode is held 
upon the back of the neck over the seventh cervical vertebrae. The cur- 
rent should be applied for five minutes at a time, three times daily, in 
the strength of from two to three milliamperes. Vigouroux employs 
the induced current, the positive electrode being placed upon the neck 
and the smaller cathode over the carotids ; this current should be applied 
once daily, ten to fifteen minutes at a time. 



EXOPHTHALMIC GOITRE 



847 



Surgical Treatment. — On account of the uncertainty attending 
operative procedures for the relief of exophthalmic goitre and the dan- 
ger by which they are surrounded, none of the various forms of opera- 
tions which are about to be mentioned should be tried until all medicinal 
and other therapeutic methods have failed, and should only be resorted 
to then when the disease is progressing and the symptoms distressing 
or dangerous. 





A.— The patient before operation. B— The same patient shortly after. 

Fig. 6.— Showing the Effect of Bilateral Resection of the Cervical Sympathetic. (Jonesco in Inter- 
national Clinics, Vol. I., 13th Series.) 



Degeneration of the heart or serious involvement of the nervous 
system are contraindications, except in the rare cases where the enlarge- 
ment of the thyroid is so great that the trachea is compressed, when a 
part of the gland should be at once removed. According to Buschan, 
the most suitable cases for operation appear to be those in which the 
enlargement of the thyroid gland has existed for some time previous 
to the development of the other symptoms. 



848 THE EYE AND NERVOUS SYSTEM 

Erom the statistics which have been compiled by Kocher and others, 
it would appear that operative measures hold out a fair chance of 
improvement or even of cure of cases of exophthalmic goitre, even after 
all medicinal measures have failed, and that the ligature of the thyroid 
arteries is equally efficacious and less serious than thyroidectomy. 

In 1896 Jonesco resected the superior and middle cervical ganglia 
for exophthalmic goitre, and the operation has been performed a num- 
ber of times since by others. Though the number of cases which have 
been operated on is small, no deaths have occurred thus far, and while 
the results obtained by this procedure are inferior to those observed 
after ligature or partial thyroidectomy, the operation is of value and 
may be used to supplement the action of either of these operations in 
the event of the persistence of symptoms, such as tachycardia, and ex- 
ophthalmus, after their performance. 

Subjects of Graves's disease bear general anaesthetics badly, so that 
whatever the operation, it is essential that it should be performed under 
local anaesthesia. 

Treatment of the Exophthalmus. — In the very early stages a light 
pressure bandage often decreases the exophthalmus, especially where 
the perfect closure of the lids is still possible ; when the exophthalmus 
is pronounced, tarsorrhaphy, or union of the edges of the lids, is indi- 
cated, not only for cosmetic reasons, but also as a prophylactic measure 
against corneal ulceration. Lateral tarsorrhaphy consists in shortening 
the palpebral fissure by uniting the edges of the lids at the canthi. 
This is accomplished by an incision into the margin of the upper 
and lower lids, which separates the skin from the cartilage, thus per- 
mitting the anterior layer containing the cilia to be everted and the 
groove between the two layers to gap ; the raw surfaces of the lids are 
then approximated by sutures. To insure a good hold on the edge of 
the lid and to prevent the sutures tearing out by the pressure of the 
globe, it is advisable to introduce the needles several millimetres beyond 
the border of the lid, and not including the latter. At times it is neces- 
sary to combine a median tarsorrhaphy with the lateral, in which event 
a narrow strip of skin is excised from the ciliary border of the upper 
and lower lids at their middle third, and the lids then united in the 
manner just described. 

Treatment of Corneal Ulceration. — Even in cases where corneal ulcera- 
tion is not present, the precaution should be taken to flush the conjunc- 
tival cul-de-sacs two or three times daily with a solution of boracic acid 
(gr. x ad f I i). When corneal ulceration is present, greater care should 
be taken in cleansing the eye, and two or three drops of a solution of 



EXOPHTHALMIC GOITEE 849 

atropine (gr. iv ad f § i) should be instilled night and morning. Hot 
compresses are of service and should be employed for one-half hour 
at a time three or four times daily. The ulcers should be carefully 
cleansed with peroxide of hydrogen (50 per cent.) and a firm compress 
bandage should be carefully applied. After the inflammation has sub- 
sided, if the central part of the cornea is occupied by an opacity, it may 
be possible to improve vision by an iridectomy. If perforation occurs 
abscission of the anterior segment of the globe or even enucleation may 
have to be resorted to. 

BIBLIOGRAPHY. 

I Gowers, Morbus Basedowii, in Handbueh der Nervenkrankheiten, Bonn, 1892, iii. 

p. 255. 

- Charcot, Memoire sur une affection caractarisee par des palpitations du coeur et 

des arteres, ia tumefaction de la glande thyreoide et une double exophthalmic 
Gazette Medecine de Paris, 1856, Nrs. 38, 39. 

Sur la maladie de Basedow. Gazette hebdomadaire, 1859, vi. 

Nouveau Cas de maladie de Basedow, heureuse influence d'une grossesse sur- 

venue pendant le cours de la maladie. Idem, 1862, p. 562. 

Maladie de Basedow. Idem, 1889, p. 188. 

Maladie de Basedow, formes frustes, physique, traitement par l'electricite. 

Gazette des H6pitaux, 1885, Nrs. 13, 15. 

Diarrhoe bei Basedow 'scher Krankheit. in Poliklin. Vortrage, Wien. 1893, 

S. 233. 

Schmidt, Jahrbuch, Bd. 229, S. 236. Idem, Bd. 223, S. 26. 

3 Maude, OZdema in Graves's Disease, The Practitioner, Dec. 1891. 

Nine Cases of Graves's Disease Ophthalmoplegia, Remarks on Lid-symptoms. 

St. Bartholomew's Hospital Report, 1892, xxvii. 

A Case of Ophthalmoplegia with Graves's Disease. Brain, Spring, 1892, p. 

121. 

Tremor in Graves's Disease. Brain, 1892, Autumn, p. 424. 

4 Griffith, Transactions of the Ophthalmological Society of the United Kingdom, 

1886, p. 6. 
3 Pain, cited by Trousseau, Clinique Med. de l'H6tel-Dieu, 3d edition. 

6 Fridenburg, Unilateral Exophthalmus. Section on Ophthalmology, New York 

Academy of Medicine, March 18, 1895. 

7 Sichels, Bull, gener. de ther., 1846, Bd. xxx. p. 349. 

8 Volkel, Ueber einseitigen Exophthalmos. Diss. Berlin, 1890. 

9 Prael, Archiv Fiir Ophth., 1857, iii. 2, 199. 

10 Mauthner, Ueber Exophthalmus. Wien. med. Press, 1878, S. 190. 

II Mooren, Ophthalmologische Mittheilungen. Berlin, 1874, S. 15. 

12 Rosner, Inaug. Dissertation, Breslau, 1875. 

13 Emmert, Archiv f. Ophthal., Bd. xvii. i, p. 218. 

14 De Giovanni, Referat aus Deutsche Med. Zeit., No. 98, p. 1143, 1889. 

15 Barella, Dissertation, Berlin, 1894. 

16 Hitscham, Wiener klin. Wochenschrift, 1894, p. 925. 

17 Mendel, Handbueh der Path. Anat. des Nervensystems, Part v. 
"Trousseau, Clinique Ophthal., 1902, April 10. 

19 Bistis, Archives d'Ophthal., July, 1903. 

20 Sattler, Griife-Samisch Handbueh, 1880. vi. p. 949. 

21 Von Grafe. Deutsche Klinik, 1864, No. 16, p. 158. 

54 



850 THE EYE AKD NERVOUS SYSTEM 

22 Sharkey, Lancet, 1890, ii. p. 877; Brit. Med. Journ., 1889, April 20. 
23 Hughlings Jackson, Trans. Ophth. Soc, 1886. 

24 Wilbrand and Saenger, Neurologie des Auges. 

25 Flatan, Deutsche Zeitschrift fiir Nervenheilkunde, xvii. p. 112. 

26 Raymond, Gaz. Med. de Paris, 1891, Nr. 26. 
27 Kocher, Correspondez-Bl.-Schweiger Aerzte, 1889. 

28 Mannheim, Der Morbus Gravesii,. Berlin, 1893. 

29 Russell Reynolds, Lancet, May, 1890. 

30 Passler, Deutsche Zeitschrift fiir Nervenheilkunde, vi. p. 210. 

31 Bruns, Anzeigen zur Zeitschrift fiir Psychiatrie, vol. lx. Part 5. 

32 Murray, Exoph. Goitre. Edinburgh Med. Journ., December, 1892. 

33 Hitschmann, Wien. klin. Wochenschrift, 1894, p. 925. 

34 Wilbrand and Saenger, Die Neurologie des Auges, vol. i. S. 47. 
33 Mannheim, Der Morbus Gravesii, Berlin, 1894. 

36 Mobius, Deutsche Zeitschrift fiir Nervenheilkunde, 1891, vol. i. 

37 Ballet, Gazette Hebd., 1888, p. 558. 

38 Lubrecht, Bemerkenswerthe Falle von Morbus Basedowii aus der Prof. Seholler 

'schen Klinik. Klin. Monatsblat. f. Augenheilk., 1890, S. 492. 

39 Buschan, Die Basedowsche Krankheit. Leipzig u. Wien, 1894. 

40 Mannheim, Der Morbus Gravesii, Berlin, 1894. 

41 Mobius, Deutsche Zeitschrift fiir Nervenheilkunde, vol. i. S. 460. 

42 Schmidt-Rimpler, NothnagePs System, 1898. 

43 Vossius, Beitrage zur Augenheilkunde, 1895, vol. xviii. 
44 Bristowe, Brain, 1886, p. 313. 

45 Warner, On Ophthalmoplegia Externa complicating a Case of Graves's Disease. 

Med. Times and Gaz., 1882, p. 540; Lancet, 1882, ii. p. 104. 

46 Jendrassik, Archiv f. Psych., 1886, xvii. p. 301. 

47 Chevalier, Thesis, Montpelier, 1891. 

48 Maude, St. Bartholomew's Hosp. Rep., 1892, vol. xxviii. 

49 Stellwag, Wiener med. Jahrst., 1869, S. 25. 
50 Fereol, Gaz. Hebd., 1889, p. 112. 

B1 Voss, Deutsche med. Wochenschrift, Aug. 13, 1903. 

62 Dyson, British Med. Journ., Jan. 15, 1887. 

53 Bruns, Auzeigen zur Zeitschrift f. Psych., Bd. lx., Heft 5. 

04 Wilbrand and Saenger, Die Neurologie des Auges, vol. i. S. 47. 

55 De Giovanni, Referat aus der Deutschen Medicin. Zeitung, 1899, No. 98, S. 1143. 

56 Buschen, Dissertation, Leipzig and Vienna, 1894. 

57 Berger, Archiv f. Augenheilkunde, 1902, xlvi. 2. 

58 Mackenzie, Edinburgh Medical Journal, 1897, p. 401. 

59 Stellwag, Wiener med. Jahrb., 1869, S. 25. 

60 Vigouroux, Ueber die Verminderung des elektrischen Hiderstandes bei Morbus 

Basedowii mit einem Zusatz von Eulenberg. Centralbl. f. Nervenheilkunde, 

1887, x. Nr. 23. 

Sur le traitement et sur quelques particularities cliniques de la maladie de 

Basedow. Progres. Med., 1887, xv. Nr. 43. 

Sur la resistance electrique consideree comme signe clinique. Progres. Med., 

1888, xvi. 3, 5. 

Le traitement electrique du goitre exopht. sa technique operatoire. Gaz. des 

Hop., 1891, Nr. 53. 

Traitement du goitre exopht. par la faradisation. Gaz des H6p., 1891, Nr. 

140, 144. 
01 Homen, Neurolog. Centralblatt, xi. S. 427. 
62 Liebrecht, Klin. Monatsbl. f. Augenheilk., December, 1890. 



EXOPHTHALMIC GOITKE 851 

63 Bramwell, Jahresbericht f. Ophth., 1888, S. 512. 

64 Freund, Deutsche med. Wochenseh., 1891, No. 8. 

65 O. Becker, Klin. Monatsbl. f. Augenheilk., 1880, S. 1. 

66 Story, Ophthalmic Review, 1883, p. 161. 

67 Rieger and Forster, Archiv f. Ophth., 1881, Bd. xxvii., iii. S. 109. 

68 Emmet, Arch, f . Ophth., xxviii. S. 203. 

69 Griffith, Trans, of the Ophth. Soc. of the U. K., 1886. 

70 Kast and Wiedbraxd, Archiv f. Psych., xxii. S. 524. 

71 Trousseau, Sur le goitre exopht. Arch gener. de med., 1862, S. 244; Gaz. Hebd., 

1862, p. 472. 

Du goitre exopht. Gaz. des Hop., 1863, Nr. 98 et 101. 

Du goitre exopht, Idem, 1864, p. 109 aussi Gaz. med., 1864, p. 180. 

Clinique medic, de l'Hotel-Dieu, Paris, Deutsch von Culmann Wurzburg, 1867, 

ii. p. 496. 

72 Vigouroux, Progres. Med., 1887, Nr. 43. 

73 Mannheim, loc. cit. 

74 Yeo, British Med. Journal, March 17, 1877. 



CHAP TEE XX. 

THE PSYCHOLOGICAL EFFECTS OF OPEEATIONS 
UPON THE EYES. 

By WILLIAM CAMPBELL POSEY, M.D. 

This subject maybe studied from two stand-points, — first, from that 
which, deals with the effect upon the mind of an operation which restores 
sight to individuals who were born blind or who became so by accident 
or disease, and, second, from that which considers the impression such 
operations produce upon the mentality in the nature of a shock, quite 
independent of any influence which might be exerted by the restoration 
of sight. Before considering either of these phases, it is necessary to 
inquire whether the mental state of the congenitally blind differs from 
that of those born with all their faculties, and to what extent loss of 
sight affects the mind of individuals who have had both mind and sight 
developed. In answer to these questions it may be stated that in general, 
blindness in no way affects the mind in its development, and the con- 
genitally blind possess as good a mentality as their fellows. It is a 
matter of experience also that subjects with healthy minds, who have 
lost their sight after their mental powers have been developed, suffer 
no impairment of reason, apart from the general depression consequent 
upon the realization of how much they have been deprived, normal 
cerebration being continued without interruption. In individuals, how- 
ever, who are intellectually weak, it can be readily understood how the 
loss of so important a sense as sight might be of serious moment, even 
sufficient, perhaps, to break the links in the fragile chain of cerebration 
and cause insanity. Xotwithstancling this, instances of serious mental 
lesions from visual defects are very rare and blindness as a causal factor 
in the production of insanity is mentioned but seldom in alienist litera- 
ture. It is probable, however, that were a more careful etiological study 
made of the blind insane from an ophthalmological stand-point, that a 
connection between the mental and ocular conditions would be traced 
more often. The cases which have been reported, however, are espe- 
cially significant, for they demonstrate that not only can insanity be 
traced to loss of sight, but that the relief of the blindness by operation 
can restore the mental condition. 



MENTAL EFFECT OF EYE OPERATIONS 853 



RELIEF OF INSANITY BY EYE OPERATIONS. 

The literature contains but few cases where it could be definitely 
ascertained that an improvement in the mental state was noted after 
such operations, for, although cataracts have been removed from the 
insane many times, either because the subjects who were operated upon 
had become chronically insane by reason of the long continuance of the 
blindness, or because their blindness was of accidental occurrence and 
in no way responsible for their mental deficiency, the mind was un- 
changed by the operation in nearly all instances. A few cases, how- 
ever, where a connection could be traced between loss of sight and loss of 
mentality have been reported, in which restoration of sight by operation 
was followed by complete recovery of mind. Thus, the writer would 
refer to two cases Avhich he reported in 1900, before the County Medical 
Society of Philadelphia. The first case, which was seen in the wards 
of the State Hospital for the Insane at Xorristown, a male, 45 years 
old, had been practically blind for 3 years with cataracts. This patient 
had inherited a disposition towards insanity from his father, who had 
had acute mania, but his mind had been apparently unaffected until his 
vision failed, when he became greatly depressed and irritable and de- 
veloped homicidal and suicidal tendencies. Rest and tonic treatment 
were prescribed without avail, the mental condition showing no improve- 
ment. The connection between the loss of sight and the insanity being 
so obvious, the cataract was removed by operation and practically nor- 
mal vision regained. The improvement in the mental condition was im- 
mediate and convalescence was so rapid that in a month the patient was 
discharged from the hospital apparently well. Shortly after, a clerical 
position was obtained, in which he has continued without relapse from 
sanity during the past six years. 

The second case occurred in a woman, 60 years of age, who had de- 
veloped melancholia, with failure of sight consequent upon cataracts 
forming upon both eyes, five years previous to the writer's first observa- 
tion of her. The mental condition was extremely bad : the patient being 
excessively violent at times and never entirely rational. Vision was 
restored by operation and was followed by a rapid and permanent return 
of a normal state of mind. Cases analogous to these have been reported 
by Bouissin and Meiser. That of the former author referred to a young 
man who had lost his mind with the development of cataract and re- 
covered it when the cataract was removed. Tn Meisor's l patient, a 
woman, 6S years old, also the subject of cataracts, hallucinations similar 



854 THE EYE AND NERVOUS SYSTEM 

to those observed after operations upon the eye developed with the 
failure of her vision hut disappeared after the removal of the cataract. 

In addition to these cases where the mental disturbance was made to 
disappear by the restoration of sight, cases have been reported in which 
the removal of peripheral irritation by enucleation of the globe was fol- 
lowed by the relief of severe mental symptoms. Thus, Moulton reports 
a case in which progressive melancholia, which had developed secondary 
to an irido-cyclitis of long standing, was immediately relieved by re- 
moval of the eyeball. Of a similar nature was a case recently reported 
by Hansell. 2 This patient, a female 25 years of age, had been born 
blind, but during her youth had given evidences of average intelligence 
and had some school training in a blind asylum, where she had later 
become a teacher. After some years she developed pain in the head 
and delusional insanity, the delusion taking the form of the envelop- 
ment of all objects in a bright red glare. The eyes were injected, 
tender to the touch, sightless and atrophic, and as it was thought that 
they might be the possible source of her delusion, both were enucleated. 
The patient gradually became more quiet and in a few days after the 
operation her mind regained its former clearness, and all delusions 
vanished, so that she was enabled to resume her occupation of teaching. 
The relief of similar symptoms by operation has been noted by De 
Schweinitz 3 and Risley. 4 

Erom these cases it would appear that grave mental disturbances may 
follow affections of the visual apparatus and that relief may be ob- 
tained by the removal of the cause, and although the writer has been told 
by alienists that many forms of insanity may be temporarily improved 
if not entirely relieved by any form of operation, or even by shock, or 
by any force which strongly impresses the mind, he believes that the 
connection between cause and effect in the cases which were reported by 
him was too close to deny the direct influence of the restoration of sight 
upon the mind. He would not deduce from this that the mind can be 
restored to all blind insane by similar operations, but he is convinced 
that every effort should be made to give useful vision to all such cases, 
for although in many instances operations may be unavailing, yet the 
return of even a few such subjects to society will amply compensate for 
many failures. 

MENTAL DISTURBANCES AFTER EYE OPERATIONS. 

This subject refers to the second category contained in the opening 
paragraph and relates to the impressions made upon the mind by the 
operation itself, independent of any influence which might be exerted 



MENTAL EFFECT OF EYE OPERATIONS 855 

upon it by the restoration of sight. Such impressions develop as a 
consequence of the shock to which the patient is subjected at the time of 
the operation and as a result of conditions which arise afterwards, and 
are of the nature described by neurologists as traumatic neuroses. 

In contradistinction to the phase of the subject which has just been 
considered, where it was shown that restoration of sight rarely exercises 
any decided action upon the mind, it not rarely happens, after opera- 
tions upon the eye, that those who are operated upon develop more or 
less disturbance of mentality. This derangement, as a rule, takes the 
form of a delirium which appears usually upon the evening of the third 
day after the operation and continues a few days to a week, according 
as the surgeon is prompt in combating it, and fortunate in his choice 
of remedies. Although frequently retarding the healing process by the 
irritation which is provoked in the eye at the height of the delirium by 
interference with the dressings, etc., the ultimate success of the opera- 
tion is not as a rule marred by its occurrence. It is rarely seen in the 
young, but it is met with in those who are well advanced in years, and, 
according to the majority of observers, especially in those who are pre- 
disposed by heredity, alcoholism, or some other cause, to mental 
aberration. 

During the performance of the operation and for the first few days 
after it, the behavior of the patients is usually all that can be desired. 
At the end of the second or third day, however, towards evening, the 
patients become restless and wish to rise out of bed, and if unrestrained 
would tear the dressings from the eyes. Rapid and violent delirium 
may supervene. Such patients usually fancy the room on fire in which 
they are confined, and would make their escape at all hazards. Not 
rarely they imagine they are subjects of persecution ; that they are being 
pursued or unjustly confined, and they demand a knife with which to 
wreak vengeance upon their oppressors. Although the symptoms 
usually are more apparent at night, the patient rarely recovers his full 
senses during the day. Hallucinations are not always present, but there 
is usually some fixed idea which predominates. Pain in the eye is but 
rarely complained of, and rise of temperature is accidental and not 
constant. After a few days the delirium subsides and the mental facul- 
ties are slowly regained without leaving any permanent mental aberrant 
processes. 

Until Dupuytren, 5 in 1819, differentiated a purely nervous type in 
non-alcoholics, all forms of delirium occurring after operations were 
thought to be due to alcohol and were considered to he instances of 
delirium tremens. This distinguished surgeon saw this nervous form of 



856 THE EYE AND NERVOUS SYSTEM 

delirium develop after a number of diverse general operations, though 
it is of interest to note that in one instance it was recorded that the 
mental symptoms appeared after the couching of a cataract. In 1863, 
Sichel 6 contributed the first article which was directed exclusively to 
the consideration of mental disturbances after operations upon the eye 
and reported eight cases of delirium following the removal of cataract in 
old people ; later in the same year monographs appeared upon the same 
subject by Lanne, 7 Magne, 8 and Borelli. 9 In his classic article upon 
operations, in the first edition of the Graefe-Saemisch Handbuch, Arlt 
refers briefly to the subject, stating that in addition to delirium tremens, 
a peculiar delirium appears during the first few days after the operation, 
chiefly in old persons of an anxious temperament, who are broken down 
in health. 

The next observation of importance was by Schmidt-Rimpler, 10 in 
1878, who asserted that mental disturbance could originate not only 
after operations upon the eye, but could develop even without an opera- 
tion in individuals whose eyes had been bandaged or who had been con- 
fined in dark rooms. In 1890 the entire subject was discussed very 
thoroughly before several French ophthalmological societies, without 
any definite conclusion being arrived at, regarding the prophylaxis or 
etiology of the disturbances, and though a number of communications 
have ajrpeared since that time, those phases of the subject still remain 
obscure. 

Erom a comparison of the mental disturbances which occur after 
operations upon the eye with those which follow other operations upon 
the body, it appears that both processes are similar, and just as the 
delirium and mental disturbances arising after operations elsewhere 
may asume a variety of forms, so the psychoses after operations upon 
the eye may vary in their manifestations, and while the picture of the 
delirium which has been given above describes the usual symptoma- 
tology, the mental disturbance may assume other forms, being influenced 
largely by the individuality of the subject affected. Thus, the time of 
the appearance of the symptoms may vary, and instead of arising within 
the first few days, the delirium may not manifest itself until the 6th 
or Tth day and may be retarded even for two or three weeks. In like 
manner the convalescence may be protracted, and the symptoms instead 
of disappearing within a few days may persist for weeks or even months. 
While a mild delirium is the rule, the nervous disturbance may take the 
form of an active mania and though relapses are very rare and the prog- 
nosis is good in most eases, the patient regaining his normal mind at 
the end of a week, in other instances the delirium passes into the deepest 



MENTAL EFFECT OF EYE OPERATIONS 857 

melancholia, and suicide may result. On account of these variations 
in the symptoms and course of the disease, Frankl-Hochwart n has 
divided these psychoses into four groups : 1. Hallucinatory mental de- 
rangement in non-alcoholics. In this class the commencement of the 
affection is usually on the first day after the operation and occurs in 
individuals ranging from 30 to 90 years of age. 2. Simple confusion 
of ideas in senile individuals. Hallucinations are not present in this 
class, and dementia not rarely follows. 3. Delirium in alcoholism. In 
this class the delirium appears earlier and subsides more rapidly. 4. 
Confusion of ideas due to inanition, occurring in extremely marasmatic 
individuals and followed by death. 

Knapp 12 has made a still more practical division of the cases into 
three clinical varieties, (a) Light cases, exhibiting but slight mental dis- 
turbance, and occurring in eccentric and odd characters, (b) those ex- 
hibiting violent mania, (c) persons of a mild melancholic disposition 
with temporary aggravations and suicidal tendencies. 

In attempting to classify cases of mental disturbance after opera- 
tions upon the eye, Schnabel 13 holds that the delirium is analogous to 
the acute delirium which is seen in the aged, though its presence in so 
many young subjects suggests important differences. Mendel 14 re- 
gards the disturbance as a form of delirium hallucinatorium, including 
under this heading, in addition to the mental symptoms after operations 
upon the eye, other traumatic psychoses as well as senile delirium. He 
insists upon the necessity of strongly differentiating other types of de- 
lirium from senile debility, for he believes that organic changes are 
rarely absent in senile debility and that the prognosis is consequently 
bad, wmereas the prognosis in other forms, which he considers as being 
purely functional psychoses, he regards as being the most favorable 
among mental disorders. 

Lowy 15 holds much the same view as Mendel, believing that the 
mental disturbance is a typical psychosis with a characteristic set of 
symptoms, and regards senile delirium as a stage of senile dementia. 
Schmidt-Rimpler 1G classifies the delirium among the paranoia, with the 
understanding that primary insane conceptions and hallucinations 
should be regarded as the chief symptom of paranoia. 

As might be expected by reason of the greater number of times the 
operation for cataract is performed upon the eyes of the aged, a class of 
individuals who are especially liable to be affected by mental symptoms 
after operations or shock of any kind, the number of times this com- 
plication is met with after the removal of the lens, is far greater than 
after any other operation upon the eye. There arc no statistics in the 



858 THE EYE AM) NERVOUS SYSTEM 

literature regarding the frequency of the disease, with the exception of 
those drawn from a summary of 24 cases, collected by the writer. Of 
these 24 cases the delirium appeared 11 times after simple extraction; 
8 times after the combined operation; 3 times after iridectomy for 
glaucoma, and twice in traumatic cases where no operation had been 
performed upon the eyes or adnexa. Sixteen of these cases were col- 
lected from the Wills Eye Hospital records and extended over a period 
in which 770 cataract extractions had been performed in that institution, 
making a percentage of 2yV per cent. It was felt by the writer, 17 how- 
ever, that these figures might be misleading and mental disturbances 
might be much more common, as the custom which prevails in that hos- 
pital of administering hypnotics at the first symptom of mental unrest 
might have prevented the development of what would have been a true 
post-operation psychosis. Of 12 cases observed by Kipp, 5 followed cat- 
aract operation, 1 an iridectomy for glaucoma, 3 traumatic injuries of 
the eyeball, and 3 corneal ulceration. Schnabel 18 saw 12 cases of delir- 
ium among 183 cases of senile cataract in his own clinic at Innsbruck, 
though in Jiiger's clinic in Vienna delirium occurred but 11 times 
among 1500 cases of cataract. All of Schnabel's cases occurred in old 
subjects and several times after iridectomy without removal of the lens. 
The psychoses began 20 hours to 6 days after the operation and lasted 
from 1 to 4 days. In half of the cases the mental symptoms occurred at 
night and in the other half during the day. Convalescence was rapid 
and without complications. Lowy explains the unusual frequency with 
which Schnabel saw cases in Innsbruck and its rarity in Vienna, by 
the fact that the inmates of the hospital in Innsbruck were, for the 
most part, inhabitants of the mountains and unaccustomed to restraint 
and confinement and more liable to be affected by such conditions than 
the city-bred Viennese. He says that the same explanation applies to 
Christen of Pavia's cases also, the frequency with which delirium 
occurred in his clinic in that city being accounted for by the unre- 
strained and rather wild character of the inhabitants of that neighbor- 
hood. 

As a rule, recovery is more rapid, the symptoms less violent and the 
prognosis generally better among the aged than the young. Webster 19 
saw a fatal case, as the result of meningitis, secondary to panophthal- 
mitis, which resulted from a patient with delirium tearing off the 
bandage. Berry 20 recorded epileptic attacks following an intraocular 
hemorrhage complicating cataract extraction. 

Etiology. — As would be expected from the nature of the affec- 
tion and our ignorance regarding the pathogenesis of psychoses in gen- 



MENTAL EFFECT OF EYE OPEKATIOXS 859 

eral, many theories have been advanced to explain the origin of the 
affection. Sichel thought the delirium was due to a change in the dis- 
position of the individual, which resembled nostalgia. A number of 
authors, however, assume a special tendency towards mental derange- 
ment in all who become delirious following operations upon the eye. 
Among these may be mentioned Arlt, 21 Schmidt-Kimpler, 22 Knies, 23 
Warloment, 24 Hirschberg, 25 and Valude. 26 These authors believe that 
mental symptoms arise in only very weak and nervous people, but fail 
to mention further characteristics of this predisposition. Lopez thinks 
that the delirium occurs only in those who are already actually mentally 
alienated, although usually in a latent form, the mental weakness having 
passed unperceived by the physician. 

Among occasional exciting causes may be mentioned Parinaud's 27 
explanation of extreme preoccupation of the patient for several days 
preceding the operation itself, and the withdrawal of all external im- 
pressions during the first few days after the operation, resulting from 
the absolute rest in bed, and the darkness and seclusion enforced by 
the closed eyes. Schmidt-Rimpler 28 is strongly of the impression that 
one of the chief factors in the production of the delirium is the ban- 
daging of both eyes, and in support of this theory cites two cases in 
which no operation was performed, but who developed delirium, as he 
thought, simply by reason of their being confined in a darkened room 
during the treatment for their ocular affection. One of these cases 
was that of a young man, 19 years old, who was suffering from irido- 
chorioiditis ; the other that of a man, 59 years old, who had plastic iritis. 
This theory is supported by Sichel, 29 Swanzy, 30 Valude, 31 Borelli, 32 
and Armaignac ; 33 the last three authors giving the notes of cases where 
the delirium disappeared with the removal of the bandage. 

Another set of authors, including Galezowski, 34 Salvator, Angela 
Ledda, and Lemkiewicz, attributes the delirium to the action of atropine. 
Grandclement 35 subscribes to this theory also, as the mental condition 
of a case observed by him was made worse after each instillation of this 
drug. 

As just mentioned, Schnabel was of the opinion that the psychosis 
was merely a form of senile delirium and he explained the transitory 
nature of the disturbance in mentality as well as its sudden appearance, 
to circulatory changes in an atrophic brain. lie thought that the hyper- 
emia of the brain is favored by the prolonged rest upon the back, with 
the stillness and darkness, to which the patients are subjected while in 
a more or less excited condition. He does not believe Schmidt-Bimpler's 
theory of the exclusion of light, as the sole cause, as he had observed 



860 THE EYE AND NERVOUS SYSTEM 

the condition to arise after the bandage had been removed, and calls 
attention to the rarity with which delirium occurs in the large number 
of young persons who are treated in darkened rooms and with bandaged 
eyes. Kraft-Ebbing 36 is of the opinion that fear is the causal factor, 
and thinks that the symptoms rarely develop in those who have normal 
minds, but rather in those who are abnormally excitable, in imbeciles, 
or in those weakened by senility, alcohol, or sickness. Frankl-Hochwart 
called attention to the connection between affections of the nerves (espe- 
cially neuralgia) and psychoses and also to the fact that mere injury 
of the globe, as in the cases reported by Griesinger, 37 Arlt, and Eiirstner, 
could occasion delirium and even death, and ascribes the delirium to an 
affection of the sensory nerves of the eye conjoined with senility, dark- 
ness, absolute rest in bed and the isolation from the external world. 

An additional theory is mentioned by Berger, 38 who says that he 
remembers that Meynert, of Vienna, ascribed the production of the 
delirium to the excision of part of the iris, which is one of the steps in 
the common operation for cataract. Berger, however, remarks that this 
theory is no longer tenable, since Parinaud has observed the delirium 
following the extraction of cataract without iridectomy. 

The restriction in diet and lessening of the amount of food imme- 
diately before and after the operation has also been advanced as a cause. 
Dupuytren was the first advocate of this theory, as he found that after 
he gave his patients sufficient nourishment, mental derangements oc- 
curred much more rarely. Parinaud and Gillett de Grandement also 
support it, for both think that the weakness induced by the restricted 
diet is an important factor in precipitating the delirium ; the latter 
author citing a remarkable instance which was evidently induced by 
this cause. 

While it is evident that in alcoholism the excitement consequent 
upon the operation, conjoined with the depressing circumstances which 
are attendant upon the after treatment, may easily provoke a delirium 
of persecution, it is not the purpose of the writer to consider this type 
of cases in this paper. In this connection, however, reference may be 
made to a case reported by Lopez, 39 where the delirium occurred in an 
alcoholic, in whom the eyes were not bandaged and where the wound 
healed perfectly. Lopez thinks that it is a circumstance Avorthy of 
consideration that he has always observed the delirium in the old and 
atheromatous, and says this has led him to think that this disease of the 
vascular system plays an important role in the production of cerebral 
troubles, and subsequently of maniacal paroxysms. Lie adds that it is 
not strange that the cause of the delirium in question should be charged 



MENTAL EFFECT OF EYE OPERATIONS 861 

Dy some to alcoholism, as this is a frequent factor in the production of 
atheromatous degeneration. 

Martin 40 also, who has contributed a recent and comprehensive 
article on the subject, holds that the delirium is due to inanition, but 
thinks that this is induced chiefly by the withdrawal of alcohol. He 
does not believe that the mental derangement in this class of cases is 
an alcoholic delirium in the sense that the subjects are ever intoxicated 
"by alcohol, but that it is delirium occasioned by the privation of this 
agent in individuals who are accustomed to it in moderate amounts; for 
he thinks that the brain, especially in the old, which has been habituated 
to act normally under the influence of alcoholic stimulation, becomes 
deranged if this agent is withdrawn. 

Fernandez 41 believes the abstinence from customary alcoholic 
stimulation is the determining factor in producing the delirium. He 
has observed the conditions six times in a very large experience, and 
only after cataract operations. 

In a paper which was presented at the Congress of Alienists of La 
Hochelle, in 1893, Regis and Chevalier-Lavaure considered the de- 
lirium which is observed after operations in general to be due to auto- 
infection as a result of uraemia and toxins originating in the intestines, 
and thought that the mental disturbance in these cases was analogous to 
those occurring in typhoid fever, influenza, and erysipelas. Fromaget 
subscribed to this theory as being operative in most cases of delirium 
after operations upon the eyes and was of the opinion that the conditions 
favoring autoinfection were found in the disturbances of the liver, 
kidney, and heart, which are common to the aged. In support of his 
views, he gave the notes of a man aged 92 years, in whom an anuria 
prevailed two days after the removal of the lens, the flow of urine being 
increased after some difficulty by injections of caffein. This theory of 
autointoxication has also been subscribed to by Finlay 42 from con- 
clusions based upon the study of a case where there were signs of 
uraemia. 

Kipp 43 in a recent article, in which he reported 12 cases of delirium 
after operation and injury of the eye, concluded from a study of these 
cases that the " psychoses were the result of a change in the patient's 
environment and to an increasing longing to get away from the new 
surroundings." He refers to the condition as a homesickness, or nostal- 
gia, which may end in a form of melancholia with homicidal and 
suicidal propensities. 

Such are the theories which have been advanced to account for this 
strange form of mental disturbance, and while in the present state of 



862 THE EYE AND NERVOUS SYSTEM 

uncertainty regarding the nature of psychological processes in general, 
it may be impossible to determine the precise etiological factor which 
occasions the delirium, there are such decided objections which can be 
offered to some of them that they may be eliminated from the list. 
Thus, the assertion that the mental aberration is due to a predisposition 
to mental unsoundness can be disproved by the absence of symptoms in- 
dicating insanity in the previous history of most of the patients, and by 
the transient character of the delirium. In the few cases in which the 
mind does not recover its normal condition after one or two weeks, but 
lapses into mania and melancholia, it is fair to suppose that some such 
conditions may have existed previous to the operation. 

Martin could satisfy himself of a state of mental weakness prior to 
the operation in but two cases mentioned in the literature, — i.e., that of 
Warloment, where the statement is made that his case seemed mentally 
" innocent," but that he had never given another sign of delirium or 
insanity ; and that of Hirschberg's, where the patient was said to have 
some mental unsoundness. He says that in none of the 20 or more 
other cases mentioned is there any reason to suspect a prior predisposi- 
tion towards insanity. Lopez's claims of a mental predisposition should 
not be regarded, as two of his cases were probably quite insane before 
the operation, and should not really have been described as instances of 
post-operative psychoses ; one of the cases occurring in a family of eccen- 
tric members and the patient remaining insane for two months after the 
operation, while the second case was melancholic. 

Sweet's 44 case belongs to the same category. This author cites an 
instance of delirium of persecution following a cataract extraction which 
was evidently excited by the fear of going blind. The patient was very 
nervous, and although there was no former history of insanity, there 
was probably a strong disposition toward mental weakness. The patient 
died two months after the operation. 

Of the 24 cases reported by the writer, there were but 3 of question- 
able healthy mentality. 

In opposition to the bandage theory is the fact that even after it is 
removed from the unoperated eye, there is usually no improvement in 
the mental condition. Thus, neither in Warloment's and Hirschberg's 
cases nor in any of those cited by the writer was there any improvement 
after the bandages were removed. Gorecki, also, and Calderon 4o saw 
delirium after cataract operation in non-alcoholics, when the eyes were 
not bandaged, and the same was true in a case observed by the author. 

This would seem to be in exact contradiction to a case reported by 
Valude, for, in this instance, as soon as the bandage was removed from 



MENTAL EFFECT OF EYE OPERATIONS 863 

a 75-year-old patient, who had developed delirium as a result of double 
iridectomy for glaucoma, the delirium ceased; and likewise a case of 
Borelli, where amelioration was almost immediate when coquilles were 
substituted for the bandage. Martin, however, disposes of both of these 
objections, or rather exceptions, as follows : Yalude's case was a simple 
coincidence, — i.e., the bandage being removed about the time that the 
other remedies which had been employed became efficacious, while 
Borelli's case was not one of true delirium, but simply one of a fixed 
idea which existed before the operation. 

It can also be argued against the bandage and exclusion of light- 
theory, that if the delirium be due to this cause, insanity should be much 
more common among the blind than is actually the case. 

The statistics of the summary, compiled by the writer in his series 
of cases, show conclusively that atropine, also, could not be considered 
as a causal factor in the production of the delirium. Cocain can also 
be eliminated. Indeed it would seem to be proven beyond question 
that the condition of the eye itself has nothing to do with the develop- 
ment of the mental alienation. 

Sichel's and Kipp's theory that the delirium was due to homesick- 
ness cannot be true in all cases, for the delirium has been known to 
originate in many cases when the patient was operated upon in his own 
home. Many of Kipp's cases seemed to be alcoholic, and the improve- 
ment noted in their condition after they left the hospital may have been 
due to the stimulation of alcohol which they then had an opportunity of 
acquiring ; or, the restraint of the hospital being removed, to the stimu- 
lus given their mind by the necessity of self-direction and responsibility. 

But three theories remain — that of excessive preoccupation upon the 
part of the patient prior to the operation, that of the withdrawal of 
food, and that of autointoxication. Of these, the latter two may be 
readily disposed of, at least in so far as the cases which came under the 
observation of the writer are concerned, for he believes that there can 
be no question of starvation in any of the cases which he reported, as it 
is his custom to make no change in the diet until 24 hours before 
operating. At this time the first preparations are made for the opera- 
tion ; the patient is bathed and the eye which is to be operated upon is 
protected by an antiseptic dressing after thorough flushing with a 
boracic-acid lotion. A light supper is then administered, which is fol- 
lowed by a laxative pill at bedtime. The following morning the patient 
partakes of a simple breakfast, and as the usual hour of operation is in 
the afternoon, a glass of milk and a cracker are given at luncheon. 
After the operation, milk is again repeated at frequent intervals. The 



864 THE EYE AND NERVOUS SYSTEM 

diet of the second 24 hours also is largely a milk one, though broths or 
beef-tea are given twice to prevent extreme constipation. On the third 
day the patient is usually allowed to get out of bed and is placed on a 
soft diet, and if the bowels have not been opened, a laxative pill is 
administered. On the fourth day liberal and supportive meals are 
prescribed. 

Similarly, he does not think that the recent theory of autointoxica- 
tion which has been advanced by Fromaget could have operated in any 
of the cases which he has cited, for care is always exercised to avoid 
any such complications. Thus it is a rule that the urine of every 
cataract patient shall be examined the morning of the day of the opera- 
tion, and care is exercised to avoid retention after operation. The 
bowels, also, if nature has not already accomplished this, are invariably 
opened on the third day by a cathartic. 

After a critical study of the entire subject the writer has finally 
arrived at the opinion that while any of the above factors, or others, 
such as the constraint of the supine position and the unusual stillness 
of the surroundings, may operate in certain cases as etiological factors, 
the chief cause of the delirium after operation upon the eyes is largely 
psychic, and agrees with Parinaud that it is due to the preoccupation 
upon the part of the patients prior to and after the operation. While 
they may appear calm and collected and submit to the operation most 
cheerfully, if one scrutinizes them closely, in most instances he will 
find that this state of quietude is assumed and that in reality there is 
great though suppressed excitement. 

The very thought of sight after the weeks and months, nay, unfor- 
tunately, in many instances, after the years of blindness, is enough to 
upset their mental equilibrium, and the desire to assist the operator 
during the operation, and hot to wince at pain or to do anything 
that might mar its success, must also be a great tax on their courage, and 
calls forth all of their power of self-control ; and then, following the 
removal of the lens, the glimpse, though momentary, which is obtained 
of the outside world, which has been so long hidden from their gaze, 
and the realization that the suspense is over, must undoubtedly occasion 
more or less psychical disturbance. 

Treatment consists in improving the state of the cerebral circula- 
tion by nitroglycerin and strychnine, and in controlling the delirium by 
hypnotics, in a supplementary diet, and in the administration of alcohol 
if the patient has been habituated to the use of intoxicants. As the 
statistics do not show any improvement in the symptoms to follow the 
removal of the bandage from the unoperated eye, this step is not to be 



MENTAL EFFECT OF EYE OPERATIONS 865 

advised, unless the wound caused by the operation be thoroughly healed. 
There is also no reason to discontinue the employment of atropine. 

Constant oversight and judicious and tactful nursing are most es- 
sential, and amelioration in the mental condition frequently follows 
the installation of a proper person by the bedside. 

BIBLIOGRAPHY. 
1 Meiser: Psychiat. u. Neurolog. Blatt., No. 1, 1897. 
2 Han sell: Meeting of the Section in Ophthalmology of the College of Physicians 

of Philadelphia, January, 1900. 
3 De Schweinitz: Ibid. 
* Risley : Ibid. 

b Dupuytren: Legons Orales de cliniq. chirurg., 1819. 
6 Sichel: Union Med., 1863, No. 1, and Annal. d'Ocul., 149, p. 154. 

7 Laune : Du delire consecutif a l'operation de la cataracte. Gazette des Hop., 

No. 57. 

8 Magne : Du delire consecutif a l'operation de la cataracte. Bull. gen. de therap., 

etc., Paris, 1863, xiv. 463; Du delir senile suivant a la suite de l'extraction de la 
cataracte. Ibid., 1865, 254-256. 
"Borelli: Giorn. d'ophth. Ital., 1863. 

10 Schmidt-Rimpler : Archiv f. Psych., 1878-79, p. 234. 

11 Frankl-Hochwart : Jahresber. f. Psych., Bd. 9, 1889. 
12 Knapp: Arch, of Ophthal., vol. xxxii., No. 4, p. 375. 
"Schnabel: Bericht des Natur. Medic-Vereins, 1883. 
14 Mendel: Berl. klin. Wochensch., 1894, No. 29. 
15 Lowy: Allegem Zeitschr. f. Psych., 1896, vol. lii., p. 166. 

16 SCHMlDT-RlMPLER : IOC. cit. 

17 Posey : Philada. Med. Journ., Sept. 15, 1900. 

18 SCHNABEL : IOC. cit. 

19 Webster: Arch. f. Augenheilkunde, xxi. p. 191. 

20 Berry: Trans. Ophth. Soc. U. K., xi. p. 152. 
21 Arlt: Graefe-Saemisch Handbuch, 1st edition. 
22 Schmidt-Rimpler : loc. cit. 

33 Knies : The Eye in General Diseases. 

2i Warloment : Annal. d'oculist., t. liv. p. 254. 

25 Hirschberg : Ein Fall von Delerium nach Glaucoma-iridectomie. Centralb. f. 

Augenh., Leipsic, 1885, ix. 1875. 
36 Valude: Revue gen. d'ophth., 1890. 
27 Parinaud: De la Soc. Franc, d'ophth., 1890, p. 126. 
^Schmidt-Rimpler: loc. cit. 
29 Sichel: loc. cit. 

30 Swanzy: N. Y. Med. Journ., xii. p. 146; Brit. Med. Journ., 1523, 1890. 
31 Valude: loc. cit. 

32 Borelli : loc. cit. 

33 Armaignac : Revue clinique d'oculist., Mars, 1881. 

34 Galezowski : Rec. d'ophth., September. 1879; Soc. Franc, d'ophth., January. 

1885. 

35 Grandclement : Lyons Med. Journ., 29, 1890. 

36 Kraft-Ebbing : Lehrbuch f. Psych., 1888. 

37 Griesinger : Pathol, der psych. Krankh.. 1871. 

38 Berger: Les Maladies des Yeux dans leur rapport avec la pathologie general, 
p. 450. 



866 THE EYE AND NEKVOUS SYSTEM 

39 Lopez: Med. Times, August, 1894; International Med. Mag., 1894. 

40 Martin : Sui de delire consecutif a l'operation de la cataracte. Jour, de Med. 

de Bordeaux, 1894, xxiv. 173, 187, 198, 206. 
"Fernandez: Arch, de oft. Hisp.-Amer., October, 1903. 
^Finlay: Arch, of Ophthal., January, 1904. 
^Kipp: Arch, of Ophthal., 1903, vol. xxxii., No. 4, 375. 
"Sweet: Delirium and Death following Cataract Extraction. Occidental Med. 

Times, Sacramento, 1894, viii. 655. 
45 Calderon : Del delerio consecutivo a la operacion de la cataracta. Rev. clin. de 

1. gosp., Madrid, 1890, ii. 241-243. 



CHAPTER XXL 

THE SUEGICAL TREATMENT OF INTRACRANIAL LESIONS 
CAUSING DISTURBANCES OF VISION. 

By CHARLES H. FRAZIER, M.D. 

LESIONS OF THE CRANIAL SINUSES AS AFFECTING 
THE CONTENTS OF THE ORBIT. 

Anatomical Considerations. — The venous circulation at the base of 
the brain through the medium of the cranial sinuses corresponds in one 
particular especially with the arterial circulation of the same region, in 
that there is a free anastomosis not only between the sinuses on the same 
side, but also between the sinuses of the opposite side of the brain. 
Therefore, a thrombus forming in one sinus may extend by continuity 
to another on the same or the opposite side. The tissues of the orbit 
are not disturbed unless there is a lesion of the cavernous sinus ; there- 
fore, it is the conditions that affect this sinus that concern us especially. 

The cavernous sinuses lie on either side upon the body of the 
sphenoid bone, and are in direct communication with one another 
through the circular or intercavernous sinus. Into the anterior portion 
of the cavernous sinus empty the ophthalmic veins, while the posterior 
portion through the medium of the inferior and superior petrosal sinuses 
is in communication with the lateral or transverse sinus. In addition 
to these the cavernous sinus has other important anastomotic relations, 
which bring the sinus indirectly into relation with extracranial struc- 
tures. Of the most importance is the so-called vein of Trolard which, 
arising in the pterygoid plexus of veins, passes, with the third divi- 
sion of the fifth nerve, through the foramen ovale and empties into the 
inferior petrosal sinus. These means of communication explain the 
relation of cause and effect between inflammatory lesions in the dis- 
tribution of the pterygoid plexus of veins (the alveoli of the inferior 
maxillary bone, the tonsil, pharynx, etc.) and thrombosis of the cavern- 
ous sinus. By way of the temperosphenoidal sinus the infectious agents 
from the skull may be carried to the cavernous sinus. Not to be for- 
gotten is the anastomosis between the terminal capillaries of the facial 
and ophthalmic veins. Thus inflammatory lesions in the distribution of 
the facial vein by a retrograde process may give rise to sinus thrombosis. 

867 



868 THE EYE AXD XEKVOUS SYSTEM 

Finally the intimate relation between the cavernous sinus and the third, 
the fourth, the first division of the fifth, and the sixth cranial nerves is 
not to be forgotten. The pressure of a swollen sinus or of an extrasinous 
exudate will account for many of the symptoms of thrombosis. As the 
sinuses have no valves, the blood may flow in either direction. To the 
absence of valves, as well as to the freedom with which sinuses on one 
side anastomose with those on the other, must be attributed the non- 
appearance of obstructive symptoms in thrombosis of some of the sinuses 
and the difficulties, therefore, attending the diagnosis. 

THROMBOSIS OF THE CAVERNOUS SINUS. 

Etiology. — The structure of the sinuses is said to favor venous 
stasis. 1 The triangular shape of the canals increases the frictional 
resistance, the walls constructed of fibrous tissue are rigid, and the dense 
bands of fibrous tissue which pass from one wall to the other prevent the 
collapse of the sinus and in themselves offer resistance to the blood 
current. Furthermore, the horizontal decubitus naturally predisposes 
towards venous stasis, so that, in cases of long-standing illness associated 
with depression of the cardiac action, the effect of this position in co- 
operation with the diminished force of the blood stream is an important 
etiologic factor. 

In general terms, thrombosis may be due to (1) systemic disturb- 
ances, (2) to traumatism, and (3) to a septic process. Systemic disturb- 
ances are observed in the very young and the very old. In the first four 
years of life one observes the marasmic thrombosis of the old writers, as 
in patients suffering from grave forms of gastro-enteritis, cholera in- 
fantum, bronchopneumonia, more rarely tuberculosis and hereditary 
syphilis, as well as in convalescents from the acute infectious fevers, 
such as scarlet fever, typhoid fever, diphtheria, and influenza. Marasmic 
thrombosis almost invariably involves the longitudinal sinus, and the 
treatment is purely medical. Both glycosuria and albuminuria are 
believed to exert a predisposing influence in the origin of thrombosis in 
people of advanced years, but it is more than likely that this relation, 
with glycosuria especially, may be accounted for by the fact that not 
uncommonly carbuncles develop in diabetic individuals. 

Thrombosis as a result of traumatism is uncommon. The sinus has 
been injured frequently in operations upon the Gasserian ganglion, but, 
so far as I know, these injuries have not given rise to any signs of inter- 
ference with the circulation of the orbit. In the absence of infection. 



Marfan, Traite Maladie de l'Enfance, tome iv. 



SURGICAL TREATMENT 869 

gunshot wounds are more likely to determine an arteriovenous aneurism 
than thrombosis of the sinus. Fracture of the base of the skull, blows 
upon the head, and punctured wounds through the orbit, occasionally 
cause thrombosis. But four cases have been reported in which the sinus 
has been injured directly without involving the skull. 

Infective thrombosis is by far the most common. The source of 
infection may be intracranial or extracranial, and the thrombus may be 
either primary or secondary. The intracranial causes include basal 
meningitis, osteitis of the sphenoid bone, and abscess together with 
thrombosis of the lateral or other sinuses. According to Germain, 2 
secondary thrombosis — i.e., extension from other sinuses — is probably 
the most common if we accept all cases. Out of one hundred and forty- 
nine cases in which an autopsy was made, in forty-three the disease orig- 
inated in the ear, causing thrombosis of all or most of the basal sinuses, 
and it is fair to assume that the lateral sinus was the one first affected. 

The extracranial sources of infection are as follows: (a) affections 
of the orbit, panophthalmitis, chalazion, blepharitis, and conjunctivitis ; 
(b) affections of the nasal cavities, — e.g., tubercular and syphilitic 
ulcerations; (c) infection of maxillary, ethmoidal, sphenoidal, and 
frontal sinuses; (d) affections of the mouth and pharnyx, including the 
tonsils, — e.g., tonsillitis, angina, diphtheria, and alveolar abscess ; 
(e) inflammatory lesions in the distribution of the facial vein, furuncle, 
cellulitis, etc. ; (/) inflammatory lesions of the scalp. Of one hundred 
and eighty-two cases collected by Dwight and Germain the source is 
given as follows : From the eye, 4 ; nose, 9 ; mouth, 14 ; face, 34, and 
lips, 2. 

Pathological Anatomy. — The thrombi of an infective thrombo- 
phlebitis are at first red and friable, but later become grayish or yellow- 
ish and of firmer consistency. The lumen of the sinus may be com- 
pletely or incompletely obliterated and the clots are more or less adherent 
to the sinus wall. The latter becomes thickened and its internal coat 
becomes lustreless and rugose. Infective thrombi should be distinguished 
easily from the cadaveric thrombi, which are blue, friable, and not- 
adherent to the sinus walls. 

Meningeal or cerebral lesions are more or less widely distributed, 
accordino- to the extent of the thrombus. If limited to the cavernous 
sinus, a localized meningitis may develop, which may become diffuse or 
may resolve itself into an abscess. On the other hand, if the thrombus is 
extensive and involves a number of sinuses, hemorrhages, usually menin- 



Boston Medical and Surgical Journal, vol. cxlvi.. No. 18. 



870 THE EYE AND KEKVOUS SYSTEM 

geal, intra-arachnoid or subarachnoid congestion, oedema of the brain, 
and intraventricular hydrops may be looked for. Large cerebral hemor- 
rhages are seldom seen, and softening of the brain when it occurs is due 
to the local stasis or the anaemia which the hemorrhage may bring about. 

Symptoms. — The symptoms of thrombosis of the cavernous sinus for 
the most part are due to obstruction of the ophthalmic veins. They are 
due also to pressure of the sinus upon the adjacent structures, to menin- 
geal irritation, and to general systemic infection. Usually within a few 
days of the onset of the disturbance of the sinus, although in some cases 
the interval is much longer, signs of obstruction make themselves 
apparent; oedema of the eyelid and conjunctivae and face, haziness of 
the cornea, choked disk, dilatation and tortuosity of the retinal veins, 
with oedema of the papillae and retinal hemorrhages, exophthalmos, due 
to the infiltration of the cellular tissue of the orbit, ptosis, which may be 
the result either of the oedema of the upper lid or of paralysis of the 
oculomotor nerve, dilatation of the pupil ; the movements of the bulb are 
restricted (Fig. 1). Pressure of the swollen sinus upon the adjacent 
structures will excite pain in the distribution of the ophthalmic division 
of the trifacial nerve and paralysis of the third, fourth, and sixth nerves. 
While in some cases but one of the cavernous sinuses is involved, in most 
instances, on account of the free communication between the right and 
left sinuses, the thrombus extends rapidly, usually within forty-eight 
hours, from one to the other, and the same clinical picture is reproduced. 
It has been observed that the development of the symptoms on the oppo- 
site side is associated with an equally rapid subsidence of the symptoms 
in the side primarily affected. 

The cerebral symptoms are constant and form a very conspicuous 
part of the clinical picture : headache which is often most intense and of 
early onset; cerebral irritation, which may be due to meningitis or 
to circulatory disturbance and is manifested by the irritable and appre- 
hensive state of the patient; vomiting, delirium, exaggerated reflexes, 
somnolence and even coma preceding death. These symptoms may be 
due to the typhoidal state or to the circulatory changes, but when the 
process has extended to the meninges, typical symptoms of meningitis, 
such as convulsions and retraction of head, may be present. This latter 
complication is rare, however, since when the process extends it is more 
likely to extend in the direction of least resistance, — namely, from sinus 
to sinus. 

To complete the clinical picture should be added the symptoms which 
are the result of the general systemic infection, fever, chills, rapid pulse 
and respirations, and so on. As one would expect, pyaemia is a very 



PLATE VII. 




Original. Showing protrusion of the eyeball, cedema of the conjunctiva, and injection of the corneal 
vessels attending obstruction of the cavernous sinus. 



SUKGICAL TKEATMENT 871 

common termination, and the case becomes further complicated by 
metastatic deposits in the lung and other organs. 

Diagnosis. — It should be borne in mind that obstruction of the 
circulation in the cavernous sinus and ophthalmic veins is not necessarily 
due to thrombophlebitis. The pressure of a tumor in the orbit or sella 
turcica or aneurisms of the carotid artery may so interfere with the 
venous circulation as to simulate thrombosis. Cellulitis of the orbit and 
phlebitis of the ophthalmic veins may produce the same effect. In the 
case of tumors the diagnosis is usually based upon the gradual onset of 
the symptoms, upon the absence of constitutional signs of infection, and 
upon the absence of cerebral phenomena. In the absence of cerebral 
phenomena it would be almost impossible to distinguish between a true 
sinus thrombosis and a phlebitis of the ophthalmic veins or orbital cellu- 
litis. In the latter case the changes in the fundus and other signs of 
obstruction would not be so marked, the condition would not spread to 
the opposite side, and headache would be less intense and more super- 
ficial. To differentiate a thrombosis of the cavernous sinus from that of 
the longitudinal or transverse sinus it should be borne in mind that 
thrombosis of the longitudinal sinus is attended with cyanosis of the 
face, with dilatation of veins leading from the great fontanelle to the 
temporal and facial veins, and with epistaxis. Thrombosis of the trans- 
verse or lateral sinus would be preceded in almost every instance by in- 
fection of the auditory canal or mastoid cells, and would be associated 
with oedema of the mastoid region, cellulitis of the neck, phlebitis of in- 
ternal jugular vein, and perhaps paralysis of the facial and acoustic 
nerves. 

Surgical Aspects. — When one considers that barely seven in every 
hundred cases of infective thrombosis of the cavernous sinus recover, it 
would seem that surgeons would have endeavored to enter the field long 
ago with the hope of reducing the mortality, and yet until recent years 
no attempt has been made. The treatment of the condition has been 
said to be preventive only, and no further consideration has been given 
the matter. The difficulties that arise once we consider seriously the 
surgical aspects and the surgical possibilities are manifest. In the first 
place, the condition itself is one which is not easy of diagnosis, and in 
many cases the diagnosis has not been made at all, or not until metas- 
tasis has occurred, or the toxgemia is so profound and the thrombosis so 
extensive that the case is regarded as desperate, and all hope of recovery 
is abandoned. Then, again, the cavernous sinus was supposed to be so 
inaccessible that, even were operative intervention considered justifiable, 
no effort would be made to reach it. 



872 THE EYE AND NERVOUS SYSTEM 

The principles to be observed in the treatment of this condition are 
recognized fully and have been applied frequently for the relief of 
thrombosis of the lateral sinus, and with a great measure of success. If 
the cavernous sinus could be approached, opened, and drained with as 
much ease and safety as the lateral sinus, the results of such intervention 
should be as good in one as in the other. It remained for Dwight, 3 in 
an operation which he performed in January, 1900, to demonstrate the 
feasibility of opening and draining the cavernous sinus in case of 
thrombosis. The case is one of sufficient moment to warrant a brief 
abstract of it. The patient, a man forty years of age, was admitted to 
the Boston City Hospital suffering from a small abscess on the right 
side of his face just above the upper lip. He had in addition all the 
symptoms of obstruction to both ophthalmic veins, more marked in the 
right than in the left side. He was in a state of profound toxaemia. 
Under ether anaesthesia the cavernous sinus was approached by the route 
recommended by Krause for resection of the Gasserian ganglion. The 
dura was opened ; the tip of the temporal lobe was lifted upward and 
backward until the cavernous sinus was felt. With a protected bistoury 
the sinus was opened, and a quantity of dark semiclotted blood gushed 
out. A strip of gauze was introduced for drainage and the wound closed. 
The results were almost immediate ; within half an hour the exophthal- 
mus almost entirely disappeared, and to a great extent the chemosis and 
oedema. His general condition improved somewhat, but he died six and 
a half hours after the operation. The lesson to be learned from this 
operation is twofold : first, that the cavernous sinus is not without the 
realm of the surgeon's knife, and, second, that an incision into the 
sinus on one side will suffice to drain the sinus on the other side. The 
fact that both sinuses have been involved is not necessarily a contrain- 
dication to operative intervention. 

While the first recorded operation aimed directly at the cavernous 
sinus, there have been cases reported in which recovery has ensued after 
indirect drainage, — that is to say, when the cavernous sinus has become 
the seat of a thrombus which has extended from the lateral sinus, the 
latter has been opened and curetted with the intention of draining all 
the basal sinuses, and in three instances the operation was successful. In 
discussing the technique of this operation three points are to be consid- 
ered, — the method of approach, the incision in the dura, and the method 
of drainage. 

The sinus will be found most accessible if approached by the tem- 
poral route : the opening in the skull may correspond precisely with the 

8 Boston Medical and Surgical Journal, vol. xlvi, No. 18. 



SUEGICAL TREATMENT 873 

one which would be made for exposure of the Gasserian ganglion. The 
difference between the two operations would consist essentially in this, — 
namely, that in the exposure of the ganglion the operation is extradural, 
whereas in the exposure of the sinus it is almost imperative to make a 
dural incision and approach the sinus in the space between the base of 
the brain and the dura. The incision in the dura should be made on a 
level with the base of the skull, the base of the brain elevated, and, once 
the sinus is reached, it should be laid open and the thrombus thoroughly 
removed with a curette. It is most important that the seat of the 
thrombus should be effectually drained, and to this end a small cigarette 
drain should be introduced. 

TUMORS OF THE OCCIPITAL LOBE. 

Tumors in this region may be considered very properly in the cate- 
gory of intracranial lesions giving rise to visual disturbances. If the 
tumor involves the occipital lobe, hemianopsia will be the only focal 
symptom ; although, if the visual tract be involved on its way to the left 
occipital lobe, there may be sensory aphasia. The exposure of tumors in 
this region may be of exceptional difficulty if the tumor is not situated 
on the surface of the brain, immediately beneath the cranial vault. 
Tumors situated on the mesial surface or upon the surface in relation 
with the tentorium are very much more difficult of access. There seems 
to be a very much greater tendency for the brain to bulge in operations 
upon the occipital than upon any other lobe, and this of itself constitutes 
a very troublesome and serious obstacle. In the first place, it makes it 
harder to retract the brain sufficiently to explore the mesial and tentorial 
surfaces, and, in the second place, it may prevent proper coaptation of 
the edges of the dural wound. 

The osteoplastic flap for the exposure of this region is quadrangular 
in shape; the anterior margin corresponds to the line of the occipito- 
parietal suture, the posterior margin should come as near as one can go 
with safety to the lateral sinus, the superior margin should be parallel to 
and not more than one-half inch from the median line, and the base of 
the flap connects the inferior extremities of the two sides. The time 
required to reflect a flap in the occipital region will be longer than that 
for any other region, because the occipital bone is considerably thicker 
than either the parietal, temporal, or frontal bone. Once the flap is 
reflected, the surface of the brain thus exposed to view should be care- 
fully examined, and, if the results are negative, a suitable retractor is 
introduced, first on the mesial, then on the tentorial surface, and a 
careful exploration, both visual and digital, is made. Because of the 



874 THE EYE AND NERVOUS SYSTEM 

tendency of the brain to bulge in this region, once the dura has been 
reflected, the manipulations necessary for exploration should be con- 
ducted as rapidly and as delicately as possible. Eough handling and 
frequent sponging will rapidly excite such oedema and congestion of the 
brain tissue as to cause an increase in volume of the affected tissue and 
greater or less protrusion of brain substance through the dural opening. 

Chronic hydrocephalus sometimes simulates brain tumor, and, if the 
posterior horn of the lateral ventricle be very much dilated, the overlying 
layer of brain tissue may be so thin as to rupture during the operation, 
and thus establish a direct communication with the ventricle. In a case 
under the author's observation this condition was found ; at first it was 
mistaken for a cyst, but further investigation showed that the cavity 
which had been opened, was the posterior horn of the dilated ventricle. 

If, when the exploration has been completed and the tumor (if 
found) removed, there be any difficulty in closing the dural wound, 
because of undue protrusion of the brain, it will be necessary to fashion 
a flap from the pericranium sufficiently large to close the defect left by 
the insufficient size of the dural flap. The pericranial flap may be 
dissected from a portion of the skull adjacent to the field of operation or 
from the segment of bone in the osteoplastic flap. When the tumor has 
not been found, or if found cannot be removed, it is better to remove the 
pericranium from the bone in the flap, since for the permanent relief of 
pressure the osseous flap should not be replaced. The wound in the scalp 
should be closed throughout with interrupted silkworm-gut sutures and 
drainage material, preferably rubber tissue, introduced through one or 
two openings made by incising the scalp obliquely one or two centimetres 
to the outer side of the margin of the flap. The drainage material may 
be removed in twenty-four hours and the sutures one day later. 

PRESERVATION OF VISION IN CASES OF BRAIN TUMOR. 

The most common cause of disturbance of vision in cases of brain 
tumor is an optic neuritis, leading to choked disk and eventually to optic 
atrophy. While optic neuritis may be due to other intracranial diseases 
as well as to extracranial lesions — as albuminuria and chlorosis — the 
most frequent source is brain tumor, and, according to Gowers, a con- 
siderable degree of neuritis is rarely due to any other cause. Optic 
neuritis is not, as was hitherto supposed, due to increased intracranial 
tension. In fact, it is rarely due to any single factor, but the most 
potent factor is the descent of a process of tissue irritation, which 
when it reaches the papilla, sets up a more intense inflammation. But 
whatever the cause, once the neuritis begins it will lead eventually to 



SUKGICAL TREATMENT 875 

choked disk and finally to optic atrophy and total and irremediable 
blindness. It is important to bear in mind that this process does not 
develop always with the same degree of rapidity, and, as a rule, the more 
rapidly it develops the more intense it becomes. In some cases months 
may elapse and the neuritis still be of a mild grade, while in others 
again it may reach a stage of great intensity within a fortnight. The 
essential points to be remembered with relation to the development of an 
optic neuritis due to brain tumors are these : first, that, if it is not 
relieved before the optic nerve becomes atrophied, permanent blindness 
will be inevitable ; and, second, that in its early stages, by the perform- 
ance of an operation easy of execution and free from danger, the neuritis 
will subside and vision be restored. In addition to disturbance of vision, 




Fig. 2.— Original. Hernia cerebri, showing the very marked protrusion which follows the removal 
of a considerable portion of the occipital bone in palliative operations for the relief of increased 
intracranial tension. 

patients with brain tumors also suffer from headache and vomiting, and 
not only the former but the latter as well may be relieved by the same 
operation. The operation which should be performed in these cases is 
the trepanation decompressive of the French, and consists essentially in 
the removal of a not inconsiderable section of bone from the cranium. 
It matters not from what portion of the cranium the bone is removed in 
so far as concerns the effectiveness of the procedure, although preference 
should be given to the parietal or temporal region rather than the occip- 
ital or frontal. The frontal region is objectionable for cosmetic 
reasons, and the occipital region because here there seems to be a 



876 THE EYE AND JNTEKVOUS SYSTEM 

tendency to the more rapid development of a hernia cerebri, and in this 
position the protrusion would be conspicuous and unsightly (Fig. 2). A 
section of bone six to eight centimetres in its various dimensions should 
be removed from the parietal or temporal region, first on one side, and 
later on the other, if after the first operation there are any signs of recur- 
rence. It is not only unnecessary but inadvisable to remove the dura, 
because of the possibility of a fungus cerebri developing should the 
wound become infected. The dura may, however, be cut in a stellate 
fashion, as recommended by Kocher, beginning in the centre of the 
opening and making several radiating incisions towards the periphery. 
When vision is beginning to fail, delay or postponement of the operation 
for any length of time is absolutely unwarrantable. In some cases it 
may be impossible at first to localize the tumor accurately, but this should 
not constitute a contraindication, and the surgeon should proceed to 
operate, if only to save the patient's vision. Needless to say, the indi- 
cations for operation are quite as positive in inoperable growths. There 
is no treatment, whether medicinal or surgical, which gives a greater 
measure of relief to patients suffering from an incurable lesion than the 
so-called trepanation decompressive. The relief from headache and 
vomiting is often immediate and permanent, and if the optic neuritis 
is not of too long duration the patient's sight will be restored. 

TUMORS OF THE CHIASM. 

In the great majority of instances the optic chiasm is injured by 
tumors taking their origin from the pituitary body, and of these a con- 
siderable proportion occur in cases of acromegaly. No attempt has been 
made to expose or remove these growths, because they are believed to be 
inaccessible. Were it not for their inaccessibility many of the tumors 
could be removed, because a large proportion of them are benign. The 
malignant tumors, probably under any circumstances, should be re- 
garded as inoperable. Owing to the firm attachments of the dura to the 
various processes at the base of the skull, an extradural operation would 
be impossible. Even though, by the temporal route, one should succeed 
in reaching the foramen lacerum anterior, which in itself is not so easy 
of access, the sella turcica and the processus clynoideus posterior would 
still have to be passed. Kiliani 4 made some observations upon the 
cadaver for the purpose of elaborating a practical method of reaching the 
chiasm, and, after trying various means of approach, he concluded that 
the frontal route was the only rational one. His operation, as practised 



4 Annals of Surgery, July, 1904. 



CEREBELLAR OPERATIONS 877 

on the cadaver, consisted in reflecting an osteoplastic flap of omega 
shape ; the base of the flap was about one-half inch behind the coronary 
snture and three and a half inches broad, its width seven inches, and 
depth five inches. A similarly shaped flap of the dura was reflected 
after the longitudinal sinns, which at this point is no larger than a small 
vein, had been ligated. Elevation of the frontal lobe exposed the optic 
nerve. 

This operation, though necessitating a very liberal exposure, need 
not be regarded as one of unusual gravity. The rapid and efficient 
methods of making an osteoplastic flap now in practice eliminate as a 
possible objection to this operation the magnitude of the flap. The suc- 
cess or failure of the operation would depend upon the ease with which 
on the living subject, not on the cadaver, the brain could be elevated 
sufficiently to expose the affected region. Though as yet I have had no 
experience, I would not hesitate in a suitable case at least to make the 
attempt to expose the tumor. 

OPERATIONS UPON THE CEREBELLUM FOR 
RELIEF OF CHOKED DISKS. 

One of the most constant symptoms of tumors of the cerebellum is 
choked disk. The appearance of certain subjective symptoms, such as 
headache and vertigo, may prompt the patient to consult a physician 
before he has noticed any disturbance of vision, but in most cases by the 
time the physician is consulted an examination of the eye-grounds will 
reveal choked disks of greater or less intensity. 

Indications for Operation. — In general it may be said that indica- 
tions for operation in cases of suspected tumor of the cerebellum do not 
differ materially from those that apply in tumors of the cerebrum. In 
both classes of cases, once the diagnosis has been made, operation should 
not be delayed for any length of time. Too much stress cannot be laid 
upon the importance of presenting the case to the surgeon at the earliest 
possible moment. Months have been spent in the fruitless administra- 
tion of antisyphilitics before operation has been considered, and in many 
cases an unwarranted amount of time has been spent in the efforts to 
establish the diagnosis beyond the peradventure of doubt or to localize 
the tumor with mathematical accuracy. As Kocher has said, there is no 
mere excuse to-day for delaying operation because the tumor has not 
been localized exactly than there would be for declining to operate upon 
a case of intracranial hemorrhage because one was unable to determine 
the exact seat of the clot. Postponement of operation should be dis- 
countenanced for several reasons. In the first place, in cases of long 



878 THE EYE AND KEKVOUS SYSTEM 

duration the patients make very poor subjects for operative intervention ; 
the operation is itself one of considerable gravity, and the condition of 
the patient should be such as to enable him to withstand its depressing 
effect. The longer the delay the larger the tumor and the less the possi- 
bility of its complete removal. What might have been a radical opera- 
tion becomes now only palliative in its effect. These arguments against 
delay apply to the treatment of tumors in general, but the presence of 
choked disks in cases of cerebellar tumors constitutes in itself a plea 
for early operation which cannot be disregarded. If too much time is 
allowed to elapse after the beginning of the choked disk, an optic neuritis 
will develop, and the time for the partial or complete restoration of 
vision will have passed. When we consider that in practically every 
case early operation, whether it be palliative or radical, in addition to 
relieving many other distressing conditions, such as headache and vomit- 
ing, will restore vision also, it is plainly our duty to consider but one 
plan of treatment — namely, the operative — and to put it into effect 
without delay. 

Anatomical Considerations. — When one takes into consideration the 
position of the lateral and occipital sinuses with relation to the only 
means of access to the cerebellum, and the plane of the tentorium cere- 
belli, he realizes at once that there are special technical difficulties in 
surgical attacks upon the cerebellum (Fig. 3). Even when one has 
removed a considerable portion of the skull below the superior curved 
line, there will be exposed to view but a relatively small portion of its 
gross surface area. Neither the upper surface nor the under surface, 
but only a portion of the posterior aspect is exposed to view by this pro- 
cedure ; whereas, in the case of the cerebrum the entire cortex, the 
mesial surface and the base, can be exposed without any difficulty. In 
addition to the larger sinuses, certain tributaries of sufficient size to 
cause when injured profuse and sometimes alarming hemorrhage, pene- 
trate this portion of the occipital bone which must be removed. The 
most constant of these, a branch of the lateral sinus, will be found about 
two centimetres to the inner side of the mastoid process. Occasionally 
one or more will be found just below the superior curved line, external 
to the occipital protuberance. 

The proximity of the pons, and especially the medulla oblongata, to 
the field of operation should be borne in mind. If in an attempt to 
sufficient exposure of the suspected area too much traction or pressure is 
made upon the medulla, the results might be fatal. 

Furthermore, the cavity of the cerebellum is very much smaller than 
that of the cerebrum, so that there is correspondingly less space in which 



CEKEBELLAE OPEKATIONS 879 

to conduct the manipulations necessary for exposure or removal of the 
tumor. In the adult skull one hemisphere of the cerebellum is contained 
in a cavity whose greatest dimension is only 10.5 cubic centimetres. In 
addition to the difficulties that are associated with operations in a space 
so small and difficult of approach, one is hampered further by the fact 
that even under normal conditions the cerebellar hemispheres are com- 




Fig. 3.— Photograph of a horizontal section of the head cut on a level with the external auditory 
meatus ; a, representing a point at the cerebellopontile angle ; b, the auditory nerve entering the in- 
ternal auditory meatus ; c, d, e, three points on the skull. Note the distance between point a and the 
points c, d, and e, as illustrating the shortest route to the cerebellopontile angle respectively. The 
shortest distance from the skull to the angle is measured along a line drawn between a and c. The 
farther away from c or the nearer to e the greater will be this distance. 

pressed in a relatively smaller space than the hemispheres of the cere- 
brum, and are so compressed that, when tension is relieved by opening of 
a dural flap, cerebellar tissue always portrudes through the opening; 
the tissues cannot be displaced or retracted, therefore, either to the same 
degree or with as much ease as can the cerebral lobes. 

Operative Technic. — Position of the Patient. — The head and shoul- 
ders should be considerably elevated, as this will to a certain extent 



880 THE EYE AND NERVOUS SYSTEM 

diminish the amount of hemorrhage. To afford greater freedom for the 
necessary steps of the operation, a suitable head-rest should be attached 
to the operating table (Fig. 4). Schede places his patients in the sitting 
posture leaning far forward. This posture, he says, diminishes hemor- 
rhage to a considerable degree, but the position is an awkward and 
difficult one in which to retain the fully anaesthetized patient. 

Incision. — The incision begins at the tip of the mastoid process on the 
affected side and runs parallel to and one centimetre above the superior 
curved line. From this point a vertical incision is made sufficiently 
long to expose the field of operation. The flap should contain the skin, 
muscle, and pericranium. If the pericranium and muscles are reflected 
together, there will be considerably less hemorrhage. 

Removal of Bone. — For various reasons it is not feasible or desirable 
to make an osteoplastic flap. The muscles and aponeurosis are thick 
enough in this region to offer adequate protection to the underlying 
structures and to make bony closure of the opening unnecessary. A 
small opening is chiselled out at a point midway between the occipital 
protuberance and the mastoid process. Here the bone is comparatively 
thin and, as Poirier says, this is the point of greatest safety. When 
completed, the opening in the skull should extend upward one centimetre 
above the superior curved line, outward as far as one can go without 
opening the mastoid cells, inward to within one centimetre of the median 
line, and downward to a point at least one centimetre distant from the 
foramen magnum. 

Exploration. — After the dural flap is reflected, one proceeds at once 
to search for a tumor, unless it has been decided to resort to the two- 
stage operation. The principles to be applied in deciding this question 
are precisely those which have been adopted in our operations for tumors 
of the cerebrum. 5 Protrusion of the cerebellum through the opening, 
once the dural flap is reflected, is indicative of increased intracranial 
tension and suggests the presence of a tumor, abscess, cyst, or internal 
hydrocephalus. Under normal conditions the cerebellum is under greater 
tension than the cerebrum, and, when the dura is incised, a normal cere- 
bellum will protrude in many cases, at least to a slight degree. The 
surface of the cerebellum should be inspected and palpated on all sides, 
to discover, if possible, any abnormality in appearance or consistency. 
If there is reason to believe that the tumor is subcortical, an exploratory 
incision is made, and, if the neoplasm is found, it is removed. On the 
other hand, if the symptoms point to a tumor at the cerebellopontile 

5 American Journal of the Medical Sciences, February, 1904. 






CEKEBELLAK OPERATION'S 



881 



angle, the subsequent steps of the operation will be attended with some 
difficulty. 

Exposure of the Cerebellopontile Angle. — To enable one to expose a 
tumor in this region it may be necessary to resort tc one of two pro- 



5v, 



.«r^ 



^-** N 




% 



Fig. 5.— The larger figure to the left illustrates the operation for the combined exposure of one 
cerebellar hemisphere and the occipital lobe of the cerebrum. The smaller figure, above and to the 
right, illustrates the structures in relation to the anterior aspect of -the cerebellum and the petrous 
portion of the temporal bone. Attention is called especially to the position of the fifth, seventh and 
eighth cranial nerves. This drawing was made by viewing the structures from the lateral aspect, 
such an exposure as would be made in exploring for tumors of the cerebellopontile angle. 1. Osteo- 
plastic flap reflected in an operation for the combined exposure of occipital lobe and cerebellum. 
2. Ninth, tenth, and eleventh cranial nerves. 3. Auditory nerve drawn to one 9ide by retractor in 
order to expose. 4. The facial nerve which lies directly beneath it. 5. The root of the trigeminus 
as it enters the groove at the apex of the petrous portion of the temporal bone. 



cedures, tapping of the lateral ventricles or removal of a large portion 
of the cerebellar hemisphere. The former method is disapproved of, on 
56 



882 THE EYE AND NEKVOUS SYSTEM 

the ground that it is so fatal in its tendencies. The alternative, on the 
other hand, is attended with very different results. The impunity with 
which large collections of cerebellar tissue may be removed, not only 
without endangering life but without disturbance of function, was an ob- 
servation made by physiologists long ago, and which has been confirmed 
in a number of cases in my own experience. When, therefore, it is dim- 
cult to expose the cerebellopontile angle, or if a tumor has been found 
and there is not sufficient space for its removal, the operator should not 
hesitate in removing at least a third of the cerebellar hemisphere (Fig. 
5). The dangers attending the manipulations necessary for removal of 
a tumor from this region, owing to the proximity of the medulla and 
pons, are not imaginary, and it is important to avoid infliction of a 

5\ - — _,6 






\J^~ V 



B* 



Fig. 6.— Operation for the simultaneous exposure of both cerebellar hemispheres, necessitating 
ligation of the occipital sinus. 1. The occipital sinus, which has been ligated previously and reflected 
with the dura. 2. Mastoid process. 3. A large tributary of the lateral sinus, invariably opened in 
cerebellar craniectomies and of varying dimensions ; said to be sometimes as large as the lateral sinus. 
4. Lateral sinus. 5. Occipital protuberance. 6. Occipital sinus. 

degree of traumatism upon these structures which may determine a fatal 
result. One should bear in mind that the shortest distance from the 
surface of the skull to this angle, and, therefore, the best method of 
approach, is along a line parallel to the petrous portion of the temporal 
bone. The region to be exposed should be approached, therefore, from 
the lateral rather than the superior or inferior aspect of the cerebel- 
lum. 'Not only is this the shortest route, but the safest also, in that 
the manipulations are carried on at a point farthest distant from such 
vital structures as the pons and the medulla. 

Simultaneous Exposure of Both Hemispheres; Bilateral Craniectomy. — J n 
many cases it will be impossible to determine beforehand whether the 



CEKEBELLAK OPEKATIONS 883 

tumor is in the right or the left lobe, so that, failing to find the tumor on 
one side, one must proceed at once, or after a proper interval of time, to 
explore the opposite side. In deciding this question one must be gov- 
erned entirely by the condition of the patient. The incision is extended 
from the termination of the point at the occipital protuberance to the 
opposite mastoid process and the flap reflected in the manner already 
described (Fig. 6). An opening in the bone is made in the same manner 
and of the same dimensions as on the opposite side and the intervening 
bridge of bone removed with a Gigli saw. The f alx cerebelli is punctured 
on either side of the occipital sinus and the latter divided between two 
ligatures. This will enable one to reflect a flap of the dura covering both 
hemispheres and to displace the cerebellum with greater freedom than 
would be possible if an intervening bridge of bone remained between the 
two openings. Bilateral craniectomy should not be resorted to as a 
routine procedure, but should be restricted to those cases in which the 
tumor is believed to occupy a position near the mesial surface or in 
which the tumor was not found on the side first exposed. In most 
instances it should be practised in two sittings. 

Palliative Operations. — Under certain circumstances we despair of 
being able to perform a radical operation : either the tumor cannot be 
found 01 cannot be localized; it may be inaccessible or it may have 
attained such proportions as to make its removal impracticable. In any 
of these contingencies a palliative operation is justifiable, and in some 
cases should be regarded as imperative. The headache, vertigo, and 
vomiting, so constant in cerebellar tumors, makes the life of the patient 
pitiable, and yet he may be relieved of all these symptoms for a consider- 
able time by adopting such measures as will relieve pressure. But the 
strongest argument against delay of operation in the treatment of cere- 
bellar tumors is the possibility of being able to save the patient's vision : 
choked disk is one of the most constant symptoms and, if permitted to 
continue too long unrelieved, an optic atrophy will develop, and the 
time for complete or even partial restoration of vision will have passed. 
Palliative operations upon the cerebellum do not consist alone in the 
removal of the overlying bone for the relief of tension, as in such opera- 
tions upon the cerebrum, but in the removal of a portion — one-third to 
one-half — of the cerebellar hemisphere. Such a procedure, mutilating 
as it seems, 'does not appear to add to the gravity of the operation, nor 
does it cause any demonstrable disturbance of function, but affords that 
relief of pressure which alone will secure the desired results. 

Hemorrhage. — Hemorrhage is not troublesome once the bone has 
been removed ; occasionally, however, there may be an alarming hemor- 



884 THE EYE AND NERVOUS SYSTEM 

rhage from one or another of the various sinuses that traverse the occip- 
ital bone. In exceptional cases these sinuses are so large that a patient 
may lose an enormous amount of blood in a very short time, so that one 
should be prepared always with suitable means for controlling hemor- 
rhage from this source. Horsley's wax should be tried first, but, if this 
fails, the outlet of the sinus should be closed with plugs of wood which 
can be whittled to the proper size and length from ordinary swab-sticks. 
Nothing but carelessness would permit of injury to the lateral sinus 
during operation. Should such an accident occur, it is quite feasible to 
control bleeding by ligation of the sinus. 

Results. — My personal experience leads me to believe that the dan- 
gers attending cerebellar operations have been somewhat exaggerated. 
I have been struck especially with the comparatively slight depression 
attending these operations and with the rapidity with which reaction 
ensues. It should be borne in mind, however, that, when the tumor is 
found, the act of removing it is not of itself attended with any additional 
risks. Mention has already been made of the fact that patients with 
cerebellar tumors are not subjects favorable for operation, yet the risk 
lies not in any feature or stage of the operation, but in the operation 
per se; therefore, the mortality attending those cases in which the tumor 
has been found and removed should not be greater than that attending 
an operation which has been solely exploratory. The mortality will be 
reduced to a minimum by observing most scrupulously those measures 
which are said to be preventive of shock : securing prompt haemostasis, 
inflicting a minimum degree of traumatism upon the cerebellar tissue, 
avoiding undue traction or pressure upon the pons and medulla, and 
resorting to the two-stage operation according to the principles already 
laid down. 

The effects of the removal of tumors, or in palliative operations of 
the removal of a portion of the cerebellar hemisphere, are as prompt as 
they are gratifying. Headache and nausea cease almost at once ; choked 
disk subsides, and vision may be almost restored or considerably im- 
proved within a few days ; the ataxia disappears and the stability of 
gait is often restored. The results of operations upon the cerebellum, 
whether radical or palliative, are usually more striking and give a degree 
of relief greater than after similar operations upon the cerebrum. The 
following statistics were compiled from one hundred and sixteen cases 
of cerebellar tumors selected by the author : 6 

6 New York Medical Journal and Philadelphia Medical Journal, February 18, 
1905. 



SUKGEKY OF FACIAL NEKVE 885 

CEREBELLAR TUMORS. 

Per cent. 

Tumors found . 45 

Tumors not found 55 

Removal with recovery 15 

Removal with improvement 13.9 

Removal without improvement 0.9 

Improvement without removal 13.9 

No improvement without removal 13.9 

Death when tumor was removed 12.9 

Death when tumor was not found and not removed 28.7 

A comparison of the statistics of successive dates shows an increase 
in the percentage of recoveries and improvements and a reduction in the 
mortality : 

Frazier's Duret's Oppenheim's 

Table, 1904. Table, 1903. Table, 1902. 

Results. Per cent Per cent. Per cent. 

Recovery 15 14 7.5 

Improved 28 25 7.5 

Unimproved •. 15 . . 13 

Mortality 42 60 71 

A comparison of the statistics of the total number of cases in 
Frazier's collection with the statistics of the last five years shows a 
manifest improvement in the results : 

Total Number Cases reported during 
of Cases. past five vears, — 

Results. Per cent. 1899-1904. "Per cent. 

Recovery 15 24 

Improved 28 28.5 

Unimproved 15 11 

Mortality 42 25.8 

SURGERY OF THE SEVENTH NERVE; OPERATIVE 
TREATMENT OF FACIAL PALSY. 

Historical. — It has "been proved hy experimental work that regenera- 
tion in a partially degenerated nerve will occur when the peripheral 
segment of the affected nerve is united to the proximal segment of a 
perfectly healthy nerve. The first application of the principles here 
involved, to the treatment of facial palsy is credited to Ballance and 
Stewart, who, in 1895, performed the first operation upon the human 
subject, uniting the facial to the spinal accessory nerve. Since that time 
this operation, modified in several ways, has been performed a number 
of times, with varying degrees of success. 

Indications. — Not every case of facial palsy presents the indications 
for the operative treatment. To prevent the method from falling hit 
disrepute, the cases should be selected with the utmost care. In the 
consideration of the treatment, cases of facial palsy for convenience sake 
may be classified as follows: 



o 



886 THE EYE AND NEKVOUS SYSTEM 

(1) Cases due to traumatism, — e.g., gunshot and stab wounds or 
fracture of the base of the skull. (2) Cases due to an infective neuritis 
secondary to mastoiditis. (3) Cases due to Bell's palsy. (4) Cases of 
paralysis following section of the nerve in the removal of a tumor from 
the face (Ballance). 

As to the traumatic cases, the sooner the operation is performed the 
better the prognosis; therefore, when the nerve is believed to be de- 
stroyed, the operation should be performed without delay. 

In those cases secondary to mastoiditis operation should be resorted 
to as soon as the infection has subsided and the possibility of wound 
infection from that source eliminated. 

Delay is not only justified but proper in all cases of Bell's palsy, but 
only for a limited time. If after the expiration of six months the 
paralysis has not responded to appropriate treatment (when the muscles 
are almost completely paralyzed and the reaction of degeneration is very 
pronounced), the operation should be performed at once. Does the opera- 
tion hold out any hope to those who have suffered from this condition for 
years — two, Hve, fifteen, twenty years or more ? In answer to this 
question it should be said that this is of comparatively little significance 
in so far as the nerve itself is concerned. The keynote to the situation is 
the condition of the muscles. If the facial muscles are completely 
atrophied and will respond no longer to galvanic stimulation, the pros- 
pects of restoration of function are extremely doubtful. Up to the 
present time seven years and nine months (Hackenbruch's case) is the 
longest period at the expiration of which the function of the nerve has 
been restored. Since the condition of the muscles plays such an im- 
portant part in the prognosis, too much emphasis cannot be laid upon 
the importance of persisting in the employment of electricity and 
massage, — i.e., such measures as will tend to preserve muscle tone. 

As contraindications to the operation may be mentioned cases of 
congenital facial palsy, provided the palsy is the result of failure of 
development of the nerve and its muscles in the medulla oblongata, and 
cases of such long duration that the muscles are so wasted that there 
is little substance capable of contraction and the nerve is so atrophied 
as to render it improbable that it could be found. 

Technic. — Before undertaking the operation two questions must 
be considered, — first, the selection of the nerve with which the anasto- 
mosis is to be effected, and, second, the method by which the anasto- 
mosis is to be made. The facial being a motor nerve, one should select 
as its anastomotic complement a motor or a mixed nerve. Two nerves 
have been proposed, — the spinal accessory and the hypoglossal. Of these 



SURGERY OF FACIAL NERVE 887 

the hypoglossal is to be preferred, chiefly because the disagreeable com- 
plication of associated shoulder movements is avoided and because the 
cortical centre of the tongue is situated nearer to the facial centre. 
After the facial nerve has been anastomosed to the spinal accessory there 
follows an association of the movements of the shoulder with those of the 
face, — that is to say, a voluntary or involuntary contraction of the 
muscles of the face is accompanied by a contraction of the muscles of 
the shoulder, and vice versa. In course of time these once conjoint 
movements become to a limited extent dissociated, — to the extent that 
the movements of the face may become independent. The patient may 
exercise control over certain groups of facial muscles independently of 
any associated movements of the shoulder, but, so far as I have been able 
to discover in the records of cases hitherto reported, there has not been 
a single instance in which a voluntary effort to move the shoulder was 
not attended with contraction of the facial muscles. These so-called 
associated movements constitute, without doubt, a very serious objection 
to the selection of the spinal accessory nerve. 

Another argument in favor of the hypoglossal nerve has to do with 
the relative positions of the cortical centres (Fig. 7). The cortical 
centre of the tongue is situated nearer the facial centre than that pre- 
siding over movements of the shoulder. Whether it is true that disso- 
ciated movements of the face are matters of education of the newly 
established cortical centre or that the impulses that precipitated those 
movements originate in the old facial centre or in the tongue centre, the 
hypoglossal nerve should be chosen. If the first or " educational" theory 
is correct, the hypoglossal nerve should be preferred because the move- 
ments of the tongue are much more closely allied with those of the face 
than those of the shoulder, and it is reasonable to infer that the newly- 
selected cortical centre could for this reason be more easily and effect- 
ually educated to perform its new function; or if the second theory 
should be proved the correct one, — viz., that the impulses must originate 
in the original facial centre, — the tongue centre has the advantage over 
the shoulder centre in that the impulses would not have to travel as far 
from the tongue to the facial centre as from the shoulder to the tongue 
centre. 

In this connection Spiller says, " In selecting the hypoglossal nerve 
we select one whose cortical centre is functionally more allied to that i)f 
the facial than is the centre of the spinal accessory, or, let us say, more 
accurately, than is the centre for the lateral movement of the head or 
elevation of the shoulder, inasmuch as movements and not muscles are 
represented in the cerebral cortex. Even if associated movements persist 



888 



THE EYE AND NERVOUS SYSTEM 



in the tongue after division of the hypoglossal, — and I cannot at present 
understand how they can do so if the motor supply of one side of the 
tongue is completely destroyed, — these movements would be hidden from 
observation, and would not cause the annoyance to the patient produced 
by spasm of the face every time the shoulder or upper limb on the same 




5TERN0-MAST0ID 
an 6 

TRAPEZIUS. 

Fig. 7.— Showing cortical origin of nerves supplying muscles to face, tongue, and shoulder. (From 
Ballance and Stewart's article on the Operative Treatment of Facial Palsy, British Medical Journal, 
May 2, 1903.) 



side is raised, after the spinal accessory has been employed for the 
anastomosis. Turning of the head or raising the shoulder is only partly 
controlled by the spinal accessory, and we can readily understand why 
an overflow into the anastomosed facial nerve occurs if the movement in 
which the spinal accessory normally has an important part is produced. 



SURGEEY OF FACIAL NERVE 889 

The cortical centre for the spinal accessory being only a part of the 
centre for turning the head or raising the shoulder, I doubt very much 
whether this centre can become so educated that it can assume perfectly 
the function normally exercised by the centre for the movements of the 
face." 

Of less significance is the question as to whether the nerve should 
be completely severed and the movements of the muscles it supplied 
sacrificed or whether only a portion of the nerve should be used to com- 
plete the anastomosis. If the spinal accessory was severed, there would 
be complete paralysis of the sternocleidomastoid and partial paralysis 
of the trapezius and drooping of the shoulder. Division of the hypo- 
glossal will be followed by paralysis of the depressors and some of the 
elevators of the hyoid bone and with atrophy of one-half the tongue. As 
compared with the deformity which it is hoped will be relieved, either 
of these disturbances may be regarded as equally insignificant. An end- 
to-end anastomosis should be preferred to a lateral anastomosis, for the 
following reasons : in the first place, the operation is easier of execution, 
if one relies on the entire nerve-trunk and not a portion or a branch with 
which to make the anastomosis ; second, because, if it is true that the 
selected cortical centre must be educated to preside over the facial 
muscles, the unlearning of its original and the learning of its new func- 
tion will be facilitated if the connection is severed completely from the 
peripheral muscle which it supplied originally and from its accustomed 
centripetal excitation ; lastly, as Spiller has pointed out, the chances of 
full restoration of power are greater after end-to-end anastomosis because 
the whole restorative force of the central end of the completely divided 
nerve is directed into the peripheral end of the facial. 7 No matter which 
view of regeneration is accepted, reunion of the divided nerves is abso- 
lutely essential to complete regeneration. If, therefore, only a part of 
the healthy nerve is brought in contact with the facial, we cannot expect 
so satisfactory results as when the entire transverse surface of the 
healthy nerve is sutured to the end of the facial stump. 

No matter which nerve is selected, the operation is not difficult 
(Fig. 8). The facial nerve is exposed by hugging closely the mastoid 

7 If from clinical observation it is proved that restoration of function will occur 
equally well after a lateral anastomosis, the latter operation may be substituted 
for an end-to-end anastomosis, providing the stump of the facial nerve is long enough 
to reach the hypoglossal nerve. Taylor recommends that the nerve be divided as far 
up in the canal as possible, in order to render available a longer section of the 
nerve, and thus make it possible to bring the facial nerve in apposition to the 
hypoglossal nerve without undue tension. 



890 



THE EYE AND NEKVOUS SYSTEM 



process and displacing the parotid gland forward and outward (Fig. 
9). The nerve will be seen entering the substance of the gland about 
one centimetre above and one centimetre to the inner side of the tip of 
the mastoid process. If the hypoglossal nerve has been chosen, it may 
be found crossing the external carotid artery just below the point at 
which the occipital artery is given off. The nerve is traced, freed, and 
finally divided at such a distance to the inner side of the external carotid 
artery as will enable the proximal end to be brought in contact with the 
peripheral stump of the facial without undue tension. The point of 
anastomosis will be upon the posterior belly of the digastric muscle. The 



& 



m 



IWoi6 P§ 



HvjpoqlojjaiN 
Occipital^ 



facia! Nerve. 



Parotid Gl. 




StPrr>o-Clei6o 
Mastoid. 



# — DujastrkM: 



ExLCarohdflf 



Fig. 9.— Author's operation for the facio-hypoglossal anastomosis. 

spinal accessory nerve may be found at the junction of the sternocleido- 
mastoid muscle and traced upward. In some instances the branch to the 
trapezius has been used instead of the main trunk. 

In making an end-to-end anastomosis three or four sutures will suffice 
to effect apposition. These should be introduced only through the sheaths 
of the respective nerves, using for this purpose the finest curved needles 
and silk. In lateral or end-to-side anastomosis, either the end of the 
divided facial may be buried within the fibres of the hypoglossal or 
spinal accessory and secured in place by sutures, or, as recommended by 
Ballance and Stewart, a longitudinal incision may be made in the sheath 



PLATE C. 




Author's operation for anastomosis of the facial with the hypoglossal nerve. 
1. Stump of the central segment of the facial nerve ; 2. Spinal accessory nerve ; 3. Reflected portion 
of the hypoglossal nerve; 4. Descendens noni ; 5. Occipital artery; 6. Internal carotid; 7. Parotid 
gland drawn forwards with a retractor ; 8. Digastric muscle ; 9. Course of hypoglossal nerve before it 
was reflected ; 10. External carotid artery. 



SURGERY OF FACIAL NERVE 



891 



of both nerves sufficient to expose the fibres, and the sutures so intro- 
duced through the sheaths of each nerve as to bring the two surfaces 
of exposed nerve-fibres into apposition. 

Clark's method effects an anastomosis by embedding the pointed 
end of the facial nerve into the substance of the hypoglossal and securing 
it there with sutures (Fig. 10). 

It is needless to say that with each of these methods success depends 
upon perfect union of the nerve, and this can only be obtained by proper 
coaptation and by providing every condition which will favor uncompli- 
cated repair of the wound. Delicacy of manipulation, use of the mini- 
mum number of sutures, perfect apposition, and complete asepsis may be 
considered absolutely essential to ultimate success. 




Fig. 10.— Clark's method of lateral anastomosis : 1, facial nerve ; 2, styloid process ; 3, internal 
jugular vein ; 4, hypoglossal nerve ; 5, pneumogastric nerve ; 6, posterior belly of digastric muscle ; 7, 
transverse process of atlas ; 8, occipital artery ; 9, sterno-mastoid muscle ; 10, parotid gland ; 11, mastoid 
process. 



Results. — There may be said to be three grades or degrees of im- 
provement, leading to complete recovery, which may be anticipated after 
the performance of this operation. The first and the least degree of 
improvement which could be expected is the restoration of muscular 
tone: the muscles are no longer flaccid; the' face in repose is sym- 
metrical, — not, as before, asymmetrical. If no greater improvement was 
anticipated, the prospect of obliterating the deformity in repose should 
settle any doubt in the mind of the patient as to the advisability of 
undergoing or in the mind of the surgeon as to the advisability of recom- 
mending the operation. The second stage or grade of improvement, and 
one which has been obtained in a majority of cases, is the voluntary 
control of individual muscles or groups of muscles, such as those con- 



892 THE EYE AND NERVOUS SYSTEM 

cerned in raising the eyebrows, in closing the lids, in puckering the lips, 
or in whistling. Such restoration is a very material gain, for not only is 
the patient's face symmetrical in repose but in action as well. The third 
or last grade, the attainment of which signifies complete recovery, is the 
return of the expressional or emotional movements, as laughing, crying, 
etc. The mechanism of these movements is much more complicated and 
a matter of delicate co-ordination. This is the ideal result, but one 
which has as yet not been obtained. Symmetry of face in repose, 
symmetry of the face in certain uncomplicated movements, and, lastly, 
symmetry in the more elaborate movements, as those of expression — 
these are the improvements aimed at in the surgical treatment of facial 
palsy. (Eor further remarks on facial palsy see Chapter VII, p. 332.) 

VISUAL DISTURBANCE DUE TO INTERNAL HYDROCEPHALUS. 

The acquired or secondary form of hydrocephalus is very frequently 
associated with disturbance of vision; in fact, optic neuritis and its 
results, choked disk and atrophy, are almost invariably present. Bitem- 
poral hemianopsia may be present, too, as a result of the pressure upon 
the middle portion of the optic chiasm exerted by the distended third 
ventricle. These conditions may be relieved only by the withdrawal of 
cerebrospinal fluid. To this end one of two methods may be resorted 
to, — Quincke's lumbar puncture and puncture of the lateral ventricles 

1. Lumbar Puncture. — Of the two operations lumbar puncture is 
the safer procedure, although the fatal tendencies of both are consider- 
able. Gumprecht collected fifteen cases of sudden death after lumbar 
puncture in cases of cerebral tumors, and of eight cases of lumbar punc- 
ture employed in cerebellar tumors four died within a few days. The 
sudden disturbance of pressure is no doubt responsible for the large 
majority of fatalities. Furbinger attributes death to pressure exerted 
upon the bulb by the arrest of cerebrospinal fluid from the ventricles 
at the foramen of Magendie, and it is no doubt especially dangerous 
when the communication between the lateral ventricles and subarach- 
noid space and the canal of the spinal cord is interrupted. Under these 
conditions the equilibrium between the cerebral and spinal fluid is 
disturbed ; the medulla and pons are drawn forcibly downward into the 
foramen magnum, with resulting traumatism, followed by serious if not 
fatal consequences. When the communication with the lateral ventricles 
is cut off, lumbar puncture will, of course, be ineffective. The results of 
this treatment for the relief of chronic hydrocephalus are on the whole 
unsatisfactory. The condition may be ameliorated temporarily by the 
relief of pressure, but only in the rarest instances will the results be 



IIS T TEEXAL HYDROCEPHALUS 893 

permanent. Gowers, for example, secured a permanent result in a case 
of serous meningitis of the chronic type ; the headache, vomiting, ataxia, 
and paralysis of the ocular muscles completely disappeared, and the 
optic neuritis subsided. Oppenheim recommends lumbar puncture for 
those cases in which the tumor is associated with an internal hydro- 
cephalus and especially when the tumor encroaches upon the posterior 
fossa and threatens the life of the patient. To withdraw fluid from the 
lower portion of the dural sac a hollow needle from two and a half to 
three inches long, armed with a stilet, is introduced between the second 
and third lumbar vertebrae. With the patient in the recumbent posture 
and the back strongly arched, the needle is introduced at a point about 
five millimetres to the right of the median line, on a level with the space 
between the second and third spinous processes. The needle is directed 
slightly inward and upward until the dural canal is reached. It is not 
necessary to aspirate the fluid, as there is enough pressure in the canal 
to force the fluid out. In children the needle may be introduced exactly 
in the median line just below the spinous process of the second lumbar 
vertebra. Whenever it is desirable to determine the pressure under 
which the cerebrospinal fluid exists, a U-shaped manometer may be 
attached by a rubber tube to the needle. The manometer may be used 
to advantage in all cases as a guide to the amount of fluid which can 
with safety be withdrawn. A pressure of twenty millimetres of water 
should be preserved. 

2. Ventricular Puncture. — Puncture of one of the lateral ventricles 
has been performed for the relief of hydrocephalus, but, as in the case of 
lumbar puncture, the effect will be only palliative. This is an operation 
which is, unfortunately, of unusual gravity, and the danger attending it 
is so great, in comparison to the possible benefit, as to make it a pro- 
cedure of questionable propriety. Many cases have been reported in 
which the results were disastrous. So far as I have been able to find, 
there has been but one instance in which the treatment was successful. 
The lateral ventricle may be entered from the frontal region, as recom- 
mended by von Bergmann, or from the parietal region, as recommended 
by Keen. By the former method a small longitudinal incision is made a 
little in front and to the inner side of the tuberosity of the frontal bone. 
A hole is bored through the skull and a long, hollow needle is introduced 
in a direction from before backward and a little downward and inward 
and pushed steadily into the brain tissue until the fluid escapes. In 
Keen's method the needle is introduced thirty-two millimetres above a 
line connecting the supraorbital edge with the external occipital protub- 
erance and thirty-two millimetres behind the external auditory meatus. 



894 THE EYE AND NERVOUS SYSTEM 



SURGERY OF THE FIFTH NERVE; TRIFACIAL 
NEURALGIA; TIC DOULOUREUX. 

In the vast majority of cases tic douloureux is a disease of the second 
and third divisions. In many cases the pain appears first in the distri- 
bution of the third division and may remain so for a long time before 
spreading to the branches of the second division. Of these two divisions 
the inferior dental and the infraorbital nerves are the branches which 
are almost invariably involved. In exceptional cases the neuralgia may 
begin in the first or ophthalmic division and never extend to the other 
branches ; on the other hand, the pain may be referred to the branches 
of all three divisions. In addition to the neuralgic pains we find in some 
cases certain points (" tender points") along the course of the nerve 
which are sensitive to pressure. Thus, when the first division is involved, 
the " tender points" will be found at the supraorbital notch, in the outer 
part of the upper eyelid, and at the emergence of the nasal branch at the 
lower edge of the nasal bone ; the pain radiates over the anterior half of 
the head, to the eyelid and eye itself, and to half of the nose ; the second 
division gives off a few branches as it traverses the sphenomaxillary 
fossa and appears at the infraorbital foramen as the infraorbital nerve, 
where it divides into the palpebral, nasal, and labial branches. When 
this division is involved, the " tender points" will be found at the infra- 
orbital foramen, at the side of the nose, and -over the most prominent 
portions of the malar bone. If the dental branches are involved, pain 
will be referred also to the teeth and gums of the upper jaw. Finally, 
if the third division is involved, pain will be referred to the distribution 
of one or all of its sensory branches ; to the parietal eminence, temple, 
and external ear (temporal and auricular temporal branch), to the 
tongue (lingual), to the gums and teeth of the lower jaw (inferior dental 
branch), and to the lower lip (mental branch). The " tender points" are 
at the inferior dental foramen, where the auriculo-temporal crosses the 
zygoma, over the parietal eminence, in the tongue, and at the mental 
foramen. 

The character and intensity of the pain are very variable. As in 
neuralgias of other nerves, the pain is spontaneous and paroxysmal ; a 
series of successive pains constitute a paroxysm and several paroxysms 
an attack. The pain is variously described as darting, stabbing, sharp, 
pricking, tearing, and boring; as to intensity, the pains vary from a 
slight burning sensation to one of excruciating severity. The attacks are 
sometimes initiated by premonitory signs, such as a feeling of tension, 
itching, or a feeling of unrest in the facial muscles, and they are pre- 



SURGERY OF TRIGEMINAL NERVE 895 

cipitated in some instances by a draught or exposure, by talking, chew- 
ing, swallowing, by fatigue and emotion. There is the widest variation 
in the frequency with which attacks recur : there may be an interval of 
only a few hours, or several months ; but usually as +ime goes on the 
attacks increase both in frequency and severity. There can be said to be 
no periodicity except in those cases in which the neuralgia is of malig- 
nant origin or in which the attacks occur only during the catamenial 
periods. (For further remarks on fifth nerve see Chap. VII, p. 320.) 

Concomitant Symptoms. — Associated with the neuralgic attacks 
there may be signs of irritation of the secretory, trophic, and vasomotor 
branches as well as of the motor root and the facial nerve. Thus, one 
may see lachrymation, flushing, or sweating (vasomotor disturbances) ; 
loss of hair and hair-pigment, and herpes (trophic disturbances) ; con- 
traction of masseter muscles (motor root), and convulsive movements 
of the face (facial nerve). The pulse is sometimes accelerated, some- 
times excited and irritable. 

Pathology. — In order to comprehend the various clinical manifesta- 
tions of trifacial neuralgia and the rationale upon which the indications 
for treatment are based, one should be familiar with at least certain 
elementary facts relating to the pathology of the affection. There may 
be said to be two or three separate and distinct types of tic douloureux ; 
one in which the lesion is primarily a neuritis of the peripheral 
branches, which in course of time may or may not extend to and involve 
the ganglion ; another in which the ganglion is affected primarily ; and 
a third, perhaps, in which the lesion is neither in the ganglion nor in its 
branches, but in the central nervous system. In some instances the 
process is a degeneration, in others an interstitial neuritis, or the primary 
lesion may be a neoplasm. Were it possible to determine by the clinical 
manifestations whether the lesion were central or peripheral, the indica- 
tions for treatment would be more clearly defined. Sometimes, how- 
ever, it is possible. According to Wagner it may be assumed that a 
neuralgia is of cerebral origin providing only several branches are in- 
volved, and providing, too, that in the other cranial nerves there is fur- 
ther evidence of cerebral disease. 

P'rognosis. — Trifacial neuralgia is of itself not a fatal disease, 
although if unrelieved the patient may from the pain and suffering 
become exhausted and more easily fall a prey to some intercurrent 
affection. In some instances the disease may be said to be self-limited 
and without any operative intervention may gradually disappear. This 
is more especially true of the so-called migrainous type, which is 
observed in women in. early life suffering from migraine. The prognosis 



MS THE EYE AND NERVOUS SYSTEM 

in the minor cases is naturally better than in those of the major or more 
severe variety. 

Classification. — Cases of trifacial neuralgia may he classified, first, 
according to whether the lesion is peripheral, ganglionic or central ; sec- 
ond, according to the distribution of the pain, whether in the distribu- 
tion of the first, second, or third divisions, in one, two or in all three 
divisions; and, third, as suggested by Dana, into two classes, one the 
migrainous type, occurring almost always in women in early life, who 
have been subject to migraine ; after a number of years it becomes 
chronic and more localized. Dana considers this type to be an evolution 
of a definite trigeminal neuralgia on the basis of a migrainous neurosis. 
In the beginning the disorder is central, neither in the Gasserian 
ganglion nor its branches, but later certain degenerative changes may 
develop in the peripheral branches. The second type of Dana's classifi- 
cation, and the more common of the two, affecting men as well as women, 
occurs after middle age, and is due to a definite lesion either in the 
ganglion or in its branches. Petres attempted to determine the seat of 
the lesion by noting the effect of an injection of a 2 per cent, solution of 
cocaine. If the injection into a painful area stops the neuralgic crisis 
for a time the lesion is presumably peripheral; if, on the other hand, 
the injection does not modify the attack the cause is higher up, either in 
the root or the ganglion. The deductions will be of value only when the 
injections are made between the paroxysms. If the paroxysms do not 
recur until the cocaine anaesthesia vanishes, it is justifiable to assume 
that the cocaine has acted in a positive manner. 

If the surgeon had some means of determining whether the pain is of 
peripheral, ganglionic, or central origin, he would at once be able to 
decide as to whether in a given case an extracranial or an intracranial 
operation were called for. As yet no means, except Petres's method, 
have been suggested whereby such a differential diagnosis may be made. 

Treatment. — The treatment may be medicinal or operative, but 
before proceeding with any definite course of treatment, one should seek 
for a cause, and, if possible, remove it. Particularly in those cases which 
are of peripheral origin may we hope to effect a cure by removing the 
source of irritation; it may be a carious tooth, a splinter of bone, a 
foreign body, a tumor, catarrhal conditions of the antrum, the ethmoidal 
or the frontal sinuses. At the onset, at least, the treatment of all cases 
of trigeminal neuralgia belongs to the domain of medicine rather than 
surgery. Clinical experience teaches us that in 20 per cent, of cases the 
disease may be arrested by medicinal measures, if not permanently, at 
least for long intervals. One must remember also that in some instances 



SURGERY OF TRIGEMINAL XERVE 897 

the disease has a tendency to run its course in five or six years and then 
exhibits a tendency to spontaneous cure. The drug which is par excel- 
lence the most efficacious, especially in the anaemic and exhausted, is 
strychnia. In cases of but one or two years' standing strychnia prop- 
erly administered will arrest or control the disease almost invariably. To 
obtain the best results the drug must be administered in heroic doses, and 
the patient should be kept under the closest observation, and should be 
confined to bed. The remedy is administered hypodermically once daily, 
in ascending closes, until, at the expiration of two weeks, the physio- 
logical limit is reached. Beginning with one-thirtieth grain the dose 
may be increased to one-fourth to one-half grain, and when the maximum 
dose is reached it should not be given oftener than on alternate days. 
Quinine, arsenic, potassium iodide, and aconitine nitrate have been 
recommended, but not one of these drugs compares in efficacy with 
strychnia. 

Extracranial Operations. — These operations include the so-called pe- 
ripheral operations and those which attack the nerves at their exit from 
their respective foramina at the base of the skull. As to the indications 
for this class of operations the general statement may be made that in 
•every case in which the pain seems to be confined to a branch of one of 
the three divisions of the ganglion a i^eripheral operation is called for. 
The simplicity of such a procedure, as compared with an operation at 
the base of the skull, whether extra- or intracranial, should deter us from 
subjecting the patient to the additional risk of more serious procedures, 
even taking into consideration the fact that there may be a recurrence. 
The tendency of the peripheral nerves to regenerate after a neurotomy 
or even a partial neurectomy should be combated by an extensive extir- 
pation of the nerves, thus reducing to a minimum the chances of recur- 
Tence. 

Given a case in which the pain may be referred to more than one 
hranch of but one division, it is proper under such circumstances to 
resort to an operation upon the nerve trunk immediately after its exit 
from the foramen. For example, in neuralgia of the second division, a 
neurectomy just above Meckel's ganglion would be called for. For those 
cases, however, in which at least two of the three divisions are believed 
to be involved one or the other of the intracranial operations should be 
Tesorted to. 

Peripheral Operations. — At one time it was thought sufficient simply 

to expose and divide the nerve. Owing to the natural tendency of the 

nerve to regenerate, recurrences were frequent. At the present time a 

peripheral operation should not be considered properly executed unless 

57 



898 THE EYE AND NEKVOUS SYSTEM 

either by excision or avulsion a considerable section of nerve is removed. 
The longer the section removed the less the likelihood of regeneration, 
and, therefore, of recurrence. 

With this idea Thiersch devised a method of forcible extraction, 
which consisted in grasping the nerve with forceps and making gradual 
traction upon the nerve both centrally and peripherally by turning the 
forceps on their own axis. For this purpose the blades of the forceps 
are so constructed that they do not cut through the nerve-fibres. One 
blade of the forceps is concave, one convex, and both are ribbed trans- 
versely or longitudinally, care being taken not to have any sharp edges. 
After the nerve is dissected free from its surroundings it is grasped at 
right angles to its axis. Traction is made very slowly, not making more 
than one-half turn per second. By this method not only may a greater 
portion of the peripheral segment even to its small terminal filaments 
be extracted, but the central segment of the nerve up to the ganglion. 

According to van Gehuchten avulsion or the forcible extraction of 
the peripheral nerves will be followed by degeneration not only in the 
ganglionic cells but also in the bulbospinal root. If this result was con- 
stant, restoration of function and recurrences of the pain would be 
impossible. Thus far, with the exception of a few cases, the results are 
based upon animal experimentation, but they are sufficiently positive to 
warrant the adoption of Thiersch and van Gehuchten's suggestion in all 
peripheral operations. 

Exposure of the Supraorbital Nerve. — To reach this nerve an in- 
cision is made parallel with the superior margin of the orbit for an equal 
distance on either side of the supraorbital foramen. The periosteum is 
divided and the nerve will be exposed to view and should be traced back 
to a point behind that at which the supratrochlear branch is given off. 

Exposure of the Infraorbital Nerve. — The infraorbital foramen 
from which the nerve makes its exit is situated about one centimetre 
below the inferior margin of the orbit and about the junction of its 
middle and inner third. A line drawn from the supraorbital notch 
downward between the two lower bicuspid teeth intersects the infra- 
orbital and mental foramina (Keen). The foramen may be exposed by 
a short incision down through the skin, subcutaneous fat and the attach- 
ment of the levator labii superioris. The periosteum is divided and 
separated below sufficiently to expose the plexus and above sufficiently 
to expose the floor of the orbit. The roof of the canal is so thin that it 
is easily broken open with a groove director. The nerve is now grasped 
with forceps and by means of traction or with the aid of the scissors 
resected back as far as the sphenomaxillary fissure. 



SURGERY OF TRIGEMINAL NERVE 899 

Exposure of the Inferior Dental Nerve. — The inferior dental nerve 
enters the canal at a point about three centimetres above the angle of the 
jaw. In order to avoid injuring the branches of the facial nerve, or 
Steno's duct, and to give the best cosmetic results, the incision should be 
made just beneath the lower border of the jaw. A musculo-periosteal 
flap, including the masseter, is reflected upwards and the canal opened 
with a chisel or trephine. In order to guard against regeneration the 
nerve should be resected as far forward as the mental foramen or 
forcibly extracted by the Thiersch method. As a further safeguard 
against recurrence the author plugs the central opening of the canal with 
some impermeable substance, as silver foil. 

Extracranial Operation at the Base of the Skull. — These operations 
are reserved for those cases in which recurrence of pain has followed 
one or more peripheral operations upon the branches of the second or 
third division, as infraorbital or inferior dental nerves, and especially 
when pain is referred to the entire area of one or the other of these two 
divisions. If both divisions are involved, an intracranial operation 
should be performed. 

Second Division. — To reach the second division at the base of the 
skull one must enter the sphenomaxillary fossa by traversing the antrum 
(Carnochan's method) or by a resection of the malar bone (Liicke's 
method). Neither of these operations is difficult of execution, although 
the latter is to be preferred; it affords more readily a better exposure 
and avoids the possibility of wound-contamination from the antrum of 
Highmore. 

Method of Liicke. — Beginning one centimetre above the outer can- 
thus and two-thirds millimetre from the external orbital margin an ob- 
lique incision is made, terminating at a point corresponding to the posi- 
tion of the upper third molar. At right angles to the first and starting at 
its upper end a second incision is made so as to expose the zygomatic 
process of the temporal bone. The malar bone is divided in the line of 
the first incision with a chain or wire saw and the zygomatic arch is cut 
through at and in front of the articular tubercle. The osteoplastic flap 
thus formed, consisting of skin, zygomatic arch, and masseter, is now 
reflected. Following the posterior surface of the upper jaw the fat and 
plexus of veins are displaced backwards ; in this way both the venous 
plexus and the internal maxillary artery may be protected from injury. 
Pressing on now, in the direction of the sphenomaxillary fossa, and 
removing as much as may be necessary to afford adequate exposure, the 
operator will reach the superior maxillary nerve at a depth of about six 
centimetres in its passage from the foramen rotundum across the spheno- 



900 THE EYE AND KEKVOUS SYSTEM 

maxillary fossa to trie infraorbital canal. Before proceeding to extract 
the nerve it must be dissected free from the infraorbital artery. It is 
then grasped with a pair of forceps, divided close to the foramen, and 
its peripheral portion extracted by the Thiersch method. Eriedlander 
resects the entire malar bone, and, while a better view is afforded, the 
operation is more radical, and there is always the possibility of opening 
the antrum of Highmore. 

Chavasse's Modification of Camochans Method^ — A horizontal in- 
cision is made below the eye and from the centre of this a vertical 
incision is dropped, exposing the infraorbital nerve. An opening an 
inch in diameter is made with trephine or chisel in the anterior wall of 
the antrum and in the posterior wall a somewhat smaller one is similarly 
made. The nerve is grasped with a pair of forceps and divided at the 
infraorbital foramen; by breaking through the floor of the orbit the 
nerve may now be traced through the antrum and sphenomaxillary fossa 
back to the foramen rotundum, where it is divided with curved scissors. 
Precautions must be taken not to injure the internal maxillary artery 
which lies in close relation with the posterior wall of the antrum. 

Third Division. — This division, with all its branches, the lingual, 
inferior dental, buccinator, and auriculotemporal nerves, may be ex- 
posed by Kronlein's method. Beginning one centimetre from the angle 
of the mouth an incision is made to a point one centimetre in front of 
the lobule of the ear. The buccinator muscle is left undisturbed, but the 
incision is carried through that portion of the masseter not covered by 
the parotid. Steno's duct and the branches of the facial nerve should 
not be injured. After separating the attachments of the masseter and 
internal pterygoid muscles from the coronoid process the latter is divided 
obliquely forward and downward and as low down as possible. The 
osteoplastic flap composed of the resected coronoid process and the 
temporal branches is reflected and the inferior dental nerve is isolated 
and traced back to the foramen rotundum, where the main trunk is 
divided. To enable one to isolate the nerve it may be necessary to 
dissect away the fatty tissue of the cheek, anterior to the masseter 
muscle, as well as the more deep-seated fat in relation with the lateral 
aspect of the internal pterygoid muscle. 

Intracranial Operations. — Division of the Sensory Root of the Gas- 
serian Ganglion; Physiological Extirpation of the Ganglion. — Upon 
the assumption that roots of the central nervous system, when divided, 
do not undergo regeneration, Spiller, in 1899, proposed division of the 
sensory root as a substitute for excision of the ganglion for the treatment 
of trifacial neuralgia. Before putting the idea into practice upon the 



SITKGEBY OF TKIGEMIKAX NEKVE 901 

human subject a series of experiments were conducted by Spiller and 
the author upon dogs, from which it was concluded that it has yet to be 
proven that regeneration of the central roots can occur. With the assur- 
ance derived from this experimental evidence the first operation was 
conducted upon the human subject in 1901. In this case the symp- 
toms were relieved, and there has been no recurrence. Since then the 
operation has been practised a number of times with equally gratify- 
ing results. 

This operation was proposed by Spiller with the hope that it would 
be found to be easier of execution than excision of the ganglion, and 
from our clinical experience it may be said that this expectation has 
been fully realized. The advantages of this procedure over any other 
having for its object the removal of the ganglion may be summed up as 
follows: (1) It is easier of execution. The most difficult step of the 
operation for excision of the ganglion is not undertaken until the attempt 
is made to free the ganglion from its base. To divide the sensory root 
the ganglion is left undisturbed once the dura propria is reflected from 
its superior aspect. All that remains to complete the operation is the 
division of the root ; thus before the most difficult stage of one opera- 
tion is even approached the other operation is finished. (2) There is less 
hemorrhage. The most annoying feature of these operations as a class 
is hemorrhage, and the most troublesome hemorrhage begins when one 
starts to elevate the base of the ganglion, since the ganglion receives its 
largest blood supply from vessels at the base. In division of the sensory 
root hemorrhage from this source does not occur because the base of the 
ganglion is not disturbed. (3) The adjacent structures are not exposed 
to injury. The proximity of the cavernous sinus, and of the third, fourth 
and sixth nerves to the inner aspect of the ganglion exposes them to in- 
jury whenever the attempt is made to extract the ganglion ; in point of 
fact the cavernous sinus has been frequently torn and one of the nerves 
divided in extraction of the ganglion. (1) Ocular disturbances, such 
as corneal ulceration, are less likely to occur. This is probably due to the 
fact that the sympathetic fibres, which pass through the ganglion, exercise 
a trophic influence on the eye. In division of the sensory root these sym- 
pathetic fibres are not disturbed. (5) The operation is easier of execu- 
tion. (6) It should be attended with a lower mortality. It may be 
assumed that of two operations the safer would be the one in which 
there is less hemorrhage, fewer technical difficulties, and, therefore, 
greater economy of time. 

Technic. — The technic of the operation is precisely the same as that 
for the extraction of the ganglion up to the point at which the superior 



902 THE EYE AND NEKVOUS SYSTEM 

and posterior aspects of the ganglion have been exposed. At this junc- 
ture the sensory root is picked up on a small blunt hook, grasped with a 
hemostatic forceps, and a small section excised. 8 

Excision of the Gasserian Ganglion. — Although it has been said that 
the ganglion may be reached readily through the occipital fossa, the best 
method of approach unquestionably is through the temporal region, as 
advocated first by Hartley and a few months later by Krause. 9 

Although the operator must be thoroughly familiar with the anatomy 
of the structures concerned, it would not be amiss to call attention here 
to some points of considerable practical importance. In the first place, 
an attempt should be made to avoid injuring the branch of the facial 
nerve which supplies the orbicularis palpebrarum. The inability to 
close the eye becomes a very serious matter after operations in which 
there is always the possibility of corneal ulceration. The incision usu- 
ally recommended for this operation is so placed that this branch of 
the facial nerve will be cut almost invariably, dishing suggested, 
therefore, that the flap be so outlined that the integrity of the nerve 
be not endangered. Second, one should be familiar with the course of 
the middle meningeal artery, and also with the fact that in some cases, 
in its passage across the anterior inferior angle of the parietal bone the 
artery is encased in a bony canal. The superior margin of the flap 
should be low enough to avoid injuring the vessel at this point. Other- 
wise the artery would inevitably be torn. This is not a serious compli- 
cation ; but would delay the operation at the most only a few moments. 
The foramen spinosum is easily identified by tracing the middle me- 
ningeal artery to the base of the skull. The position of this foramen 
is important on account of its relation to the ganglion and its branches. 
The foramen lies a little external to the posterior aspect of the ganglion 
and a little behind and external to the foramen ovale through which 
passes the third division. It is, therefore, an important landmark, and 
a knowledge of its whereabouts will be especially helpful to those who 
have had limited experience in this field of surgery. Finally, mention 
should be made of the intimate relation of the third, fourth, and sixth 
cranial nerves, as well as of the cavernous sinus, with the internal 
aspects of the ganglion. 



8 The early and subsequent stages of the operation are described in the technic 
for excision of the ganglion. 

9 No one should undertake the operation for the first time upon the living sub- 
ject. The operation should be repeated several times upon the cadaver in order that 
at least some of the difficulties may be appreciated and a vivid impression of the 
exact anatomical relations acquired. 



SURGERY OF TRIGEMINAL NERVE 903 

The Operation. — A horseshoe-shaped incision is made with its base 
corresponding to the level of the zygoma and its summit three or four 
centimetres above. The musculo-cutaneous flap is reflected and the 
zygoma resected. The latter step is important, as it enables the operator 
to approach the ganglion from a plane on a level with the base of the 
skull ; the nearer one is to the base of the skull the shorter the distance 
to be traversed before the ganglion is reached. With mallet and gouge 
an opening is made in the skull and then further enlarged with rongeur 
forceps until the opening measures three or four centimetres in diam- 
eter. With a blunt instrument, as the handle of a knife, one then proceeds 
to separate the dura from the base of the skull, following in a general 
way the direction of the petrous portion of the temporal bone. At this 
stage of the operation hemorrhage may be a troublesome though never 
an alarming feature. The firmer the adherence of the dura to the skull, 
and, as a rule, the older the patient the freer will be the hemorrhage. 
Thus it may be necessary from time to time to stop to control hemor- 
rhage by packing firmly with strips of gauze and allowing the same to 
remain in situ for one to three minutes. 

When the foramen spinosum is reached the operator separates the 
dura anterior to the foramen until the second and third divisions are 
exposed as they pass into the foramina rotunda and ovale, respectively. 
The field of operation during this and the subsequent stages of the opera- 
tion can only be exposed to view by elevating the tempero-sphenoidal 
lobe, and for this purpose a suitable retractor should be provided. The 
exposure of the ganglion is never difficult, although hemorrhage may 
be so free as to necessitate one's proceeding rather slowly. The most 
difficult step of the operation is now approached. To thoroughly iso- 
late the ganglion it must be separated from its dura propria ; to accom- 
plish this an incision is made with an Allis director or other blunt 
instrument into the dura from the second to the third division, and 
by blunt dissection the superior aspect of the ganglion is exposed. 
After dividing the second and third divisions the ganglion is sepa- 
rated from its base, grasped with a pair of forceps and forcibly ex- 
tracted. In doing this the cavernous sinus and one of the contiguous 
cranial nerves have been torn. Hemorrhage from the lacerated sinus 
may be controlled by packing, and if the middle meningeal artery has 
been injured near the foramen spinosum bleeding may be checked by 
ligating the vessel by plugging the foramen or by gauze packing. The 
wound is irrigated with normal saline solution and a strip of rubber 
tissue introduced for drainage. Certain precautionary measures should 
be adopted to protect the eye and prevent corneal ulceration. Inime- 



904 THE EYE AND NERVOUS SYSTEM 

diately before or after the operation a Buller bandage should be applied 
and the eye irrigated twice daily with a warm boracic solution. In addi- 
tion to this Keen sews the eyelids together before the operation is begun, 
and does not remove the stitches until the third day. The immediate 
results of the operation will vary considerably according to the experi- 
ence of the operator. There is no field of surgery for which there is a 
greater justification in specialization than in the surgery of the central 
nervous system. The mortality of Gasserian ganglion operations has 
been estimated as 12 per cent. ; but there is no doubt that with greater 
attention to those requirements of technic which have been elaborated 
only in recent years, and especially in the hands of those who have had 
exceptional opportunities, the mortality will be reduced. 

Abbe's Method. — As a substitute for excision of the ganglion Abbe 
recommended the forcible extraction of the second and third divisions 
and the interposition of a piece of gutta-percha tissue one and one-half 
inches by three-quarters of an inch to prevent degeneration. His 
technic differs somewhat from that already described in that he prac- 
tises preliminary ligation of the external carotid artery and exposes the 
bone by a vertical incision over the middle of the zygoma. Two objec- 
tions to this method of treatment at once suggest themselves: the first, 
that the operation is applicable only to those cases in which the pain is 
distributed only in the second and third divisions ; the second that there 
is a possibility of the rubber tissue acting in the role of a foreign body 
setting up a reaction in the tissues, which would terminate in abscess 
formation and necessitate the removal of the foreign material. Abbe 
says : " It is certainly past dispute that there is no need for the removal 
of the first branch of the fifth pair in any case of grave tic douloureux 
unless the origin is to be found in a tumor of the Gasserian ganglion or 
behind it." This may apply to the majority of cases, but it certainly is 
not a rule without exception. In fact, in two cases under my own obser- 
vation the pain was most intense in the distribution of the first division. 
It might also be said of Abbe's operation that it offers no assurance 
against subsequent involvement of the first division, and, so far as the 
mortality is concerned, it should be no less than that following division 
of the sensory root, since both are intracranial operations, the only 
difference being that in one two peripheral branches are divided, while 
in the other one divides the central root of the ganglion. 

Osmic Acid Injection. — While Billroth and Neuber were the first to 
employ osmic acid in the treatment of trifacial neuralgia, they used 
repeated injections in the skin without exposing the nerve. Burnett 
(1899) was the first to recommend the more careful intraneural injec- 



SURGERY OF TRIGEMINAL XERVE 905 

tions into the exposed nerve, and more recently (1904) Murphy has 
become an advocate of this mode of treatment. Up to the present time, 
however, it has not been proven that the results are much better than 
would be expected after an excision of the nerve trunk. The injection 
of osmic acid no doubt makes it more difficult for the nerve to undergo 
regeneration, but as yet we have no assurance that regeneration will not 
eventually occur. Besides, cases have been reported 10 in which the 
treatment was a failure. The immediate results, as regards the harm- 
lessness, and at least the temporary relief, might be said to justify the 
use of this treatment as a substitute neurectomy, before resorting to pro- 
cedures of such severity as intracranial operations. It can in no wise, 
however, be regarded as a substitute for the intracranial operations in 
those cases of major tic in which the pain is widely distributed. As to 
the technic, a one and one-half to two per cent, solution of osmic acid 
should be injected in many places into the exposed nerve trunk and also 
into the foramen. Murphy has injected the palatine, mandibular, 
lingual, supraorbital and infraorbital branches. The injections should 
be made under general anaesthesia, and all of the branches, except the 
supraorbital, can be exposed through mouth incisions. 

10 Wright, Medical Chronicle, Fourth Series, vol. vi. 



CHAP TEE XXII. 

TEEMOES ; EEFLEXES ; GAITS. 

By JOSEPH SAILER, M.D. 

TREMORS. 

By tremor we mean an oscillatory movement of some part of the 
body, occurring as a result of more or less rhythmical muscular con- 
tractions, which alternate in opposed groups of muscles. 

Carefully to be excluded are all the forms of pseudotremor pro- 
duced by rhythmical shocks occurring within the body, such as Musset's 
sign in aortic regurgitation, certain movements occurring as a result 
of aneurism, and movements in the extremities which are the result 
of vascular pulsation. There is some doubt as to whether fibrillary 
contractions within a certain muscle or group of muscles which do not 
produce movements at any of the joints, or waves of contraction passing 
along the muscles, should be included under tremors or not. 

Tremors are not necessarily signs of morbid conditions. They 
may occur as a result of certain physiological states. Thus, tremors 
of the extremities of more or less severity occur during and after pro- 
longed severe exertion, in the course of violent emotional disturbance, 
after chilling, the most frequent example being chattering of the teeth, 
after excesses of various kinds, and during convalescence from severe 
infectious disease. They may also be produced voluntarily, either for 
purposes of simulation or experimentation. In all these forms the 
movement is slow and rhythmical, and the duration is short. The head 
and limbs are the parts most commonly involved, and the movements 
are at least partially under the control of the will. 

Among the causes of pathological tremor may be mentioned par- 
ticularly the poisons. Alcohol, especially chronic alcoholism, is apt 
to be associated with a fine tremor of the hands, lips and tongue. 
Tobacco is said to cause a fine tremor of the hands and eyelids. Lead, 
mercury, anilin, and various other poisonous substances, may also pro- 
duce tremors. These are nearly all fine, slow tremors, often increased 
by voluntary movement, and affecting chiefly the upper extremities 
and the muscles of the face. In the course of various diseases of the 
nervous system characteristic tremors occur that aid materially in the 
diagnosis. 
906 



TREMORS 907 

CLASSIFICATION. 

Tremors are classed, first, as rhythmical and arhythmical. The 
rhythmical tremors frequently show in addition a rhythmical increase 
and decrease in their severity. We also speak of fine and coarse tremors. 
In the former the oscillation is very slight, perhaps imperceptible ; in 
the latter it is of greater amplitude. Tremors may be regarded as rapid 
when the rate of oscillation is from eight to twelve times per second, 
and slow when it is less than five times per second. Intermediate forms 
also occur. Tremors are spoken of as static if they are present during 
rest, and as movement or intention tremors if they are either produced 
or increased by voluntary movements. If the tremor resembles some 
familiar motion it is usually called by that name, as the tremor of 
negation when the head is rotated rhythmically from side to side ; the 
nodding or salaam tremor when the head oscillates forward and back- 
ward, and the pill-roller's tremor of paralysis agitans. 

Numerous efforts have been made to obtain some information re- 
garding the nature of tremors by means of recording apparatus. These 
all consist essentially of a moving surface and some transmitting mech- 
anism by means of which the oscillations are recorded. It has been 
possible by these methods to determine the rhythm, the rate, and, in 
a relative manner, the amplitude of the oscillations, but practically 
nothing more, and the studies have not contributed materially to our 
knowledge of disease of the nervous system. 

It will be most convenient to discuss briefly those tremors which 
occur in association with nervous disease, and are therefore of more or 
less diagnostic significance. There is one condition in which the tremor 
is practically the only sign of a morbid process. This is the simple 
hereditary or congenital tremor. It occurs sometimes sporadically, 
sometimes in several members of the same family, and occasionally 
exists in successive generations. The tremor is usually rather slow in 
rate and fairly rhythmical. It may affect almost any part of the body, 
but as a rule the head or upper extremities are particularly involved. 
The tremor sometimes develops only during activity or emotional stress, 
but often continues during rest, and indeed may be most pronounced 
during rest. Occasionally, but not often, it is under voluntary control. 
It may possibly be regarded as a stigma of degeneration, if this term 
has any real significance, but it is often found in intellectual persons, 
and even when it involves the upper extremities, does not appear to 
interfere with occupations requiring delicate manipulations. Usually 
it does not develop until adolescence, or adult life, but occasionally it 
is present in infants (Raymond). 



908 THE EYE AND NERVOUS SYSTEM 

Senile Tremor. — In old age tremor frequently develops. It usu- 
ally affects the hands, and most commonly is in the form of flexion and 
extension at the elbow and metacarpophalangeal joints, although other 
parts are frequently involved. It often affects the head, giving rise to 
a nodding movement. It is usually static; the movements are slow, 
not often exceeding live per second, and usually less than five. Volun- 
tary motion often abolishes the tremor for a short time. 

The tremor of paralysis agitans is characteristic. In addition to 
the rigidity of the muscles and peculiar attitude and expression which 
constitute the essential signs of the disease, there occurs, with very few 
exceptions, a fine static tremor of the fingers, which, in its most charac- 
teristic form, consists of opposition and withdrawal of the thumb and 
flexion and extension at the metacarpophalangeal joints. The hand, 
therefore, assumes a position somewhat similar to that seen in the spasm 
of tetany, and the movements of the fingers are supposed to resemble 
the movements made in rolling pills, hence the name, " pill-roller's 
tremor." The tremor, however, often involves the arms or head, and 
even the trunk. Its most characteristic peculiarity is that it is abolished 
or markedly diminished by voluntary movements, although upon con- 
tinuance of voluntary movement, such as writing, the tremor soon re- 
appears. 

The tremor of multiple sclerosis is the most typical of all forms of 
movement of intention tremors. It develops usually only when the 
patient makes a voluntary movement, becoming more and more violent 
until the movement is completed, when it ceases. The oscillations are 
coarse, irregular, arhythmic, and slow, ranging from one or two to five 
or six per minute. In many respects they resemble the movements of 
ataxia, and no sharp distinction can be drawn between them. It may 
be said, however, that, as a rule, the tremor of multiple sclerosis is less 
under the control of the patient, even when guided by vision, than is the 
tremor of ataxia. The symptom can best be elicited by having the 
patient grasp at an object held at arm's length from him, or by asking 
him to carry a glass of water to the lips, or by directing him to touch 
the tip of his nose with his finger. 

The tremor of exophthalmic goitre resembles most closely the toxic 
tremors. It usually occurs in the hands, and is only elicited when the 
muscles are made tense by extreme extension of the fingers. The oscil- 
lations are of small amplitude, rhythmical, rapid, and. cease only when 
the muscles are relaxed. 

The tremors of hysteria and allied functional conditions of the 
nervous system are of various types. The patient may exhibit a fine 



TREMORS 909 

constant tremor of the extremities and head, not much affected by move- 
ment, nearly always disappearing during sleep, slightly arhythmic and 
moderately rapid. The tremor is frequently unilateral, and is to be 
distinguished as hysterical only by the presence of the other stigmata 
of that condition. In common with all hysterical stigmata, it may be 
temporarily or permanently abolished by some profound emotional 
shock. Hysterical patients may also manifest from time to time irreg- 
ular waving motions, sometimes tremor-like, but often so slow that they 
cannot properly be included under this designation. They may resemble 
gestures, and occasionally have a distinctly histrionic character. They 
often have, in addition, a rapid, violent tremor that may be produced 
by different attitudes, movements, or even associations. This tremor 
may affect one extremity, the side, or perhaps the whole body. The 
movements are rhythmic, rapid, and of considerable amplitude. They 
usually increase in violence until the particular action that causes them 
to cease is applied. This may be either the assumption of some attitude 
on the part of the patient, pressure upon the trembling extremity, or 
some other purely suggestive influence. Occasionally, however, the 
tremor increases until it becomes actually a clonic convulsion, or it may 
terminate in a hysterical crisis. Tremors of this type resemble very 
closely the clonic spasms of various conditions, particularly epilepsy. 

The third group are the imitation tremors. Krafft-Ebing called 
particular attention to the frequency with which hysterical patients 
simulate the tremor of paralysis agitans, and various other types of 
tremor in organic disease are also imitated more or less closely. It 
seems to be common to all these tremors that they are more in evidence 
when the patient is under observation than when ajDparently alone ; that 
their amplitude is greater than the real organic tremors, that they are 
not likely to be rhythmic ; and that they do not cease as a result of the 
conditions that cause the cessation of the organic tremors. 

In neurasthenia a fine tremor of the extremities, static in character, 
may often be detected. In epilepsy a tremor of the hands has been 
noted during periods of disturbance of consciousness that replace the 
convulsive attack. 

In general paresis there is a fine tremor of the tongue and lips 
and occasionally a slight tremor of the hands. In hemiplegia and in 
other forms of spastic paralysis tremors are very apt to occur. They 
are usually coarse, irregular, and sometimes closely simulate the move- 
ments of ataxia. They are nearly always of the movement or intention 
type, and are exaggerated by emotional disturbances. Often with a 
return of power the tremor gradually improves and may ultimately 



910 THE EYE A^"D KEKVOUS SYSTEM 

entirely cease. Occasionally a very fine tremor persists in the side that 
has been paralyzed, even after an apparent complete return of power. 

Akin to tremors are the choreiform movements, the movements that 
occur in ataxia, and various types of clonic spasm, as, for example, ankle- 
clonus. Their description, however, will be found in other sections of 
this book. 

REFLEXES. 

By the term " reflex" we understand some manifestation of vital 
activity that occurs as a result of obvious stimulation. Possibly all 
forms of vital activity are the result of stimulants of one kind or 
another, and therefore, properly speaking, reflexes, but unless the stim- 
ulus can be detected we are not justified in using this term. Clinically, 
we make a distinction between the psychical and the mechanical re- 
flexes. By the former we mean the various actions that occur as a 
result of the stimulation of the perceptive centres of the brain, of which 
an example is the secretion of the saliva at the sight of food. Such 
reflexes have to do rather with psychology than with clinical medicine, 
and will not be further considered in this article. 

By a mechanical reflex we understand the involuntary contraction 
of a muscle or group of muscles as a result of the stimulation of certain 
sensory fibres. This definition implies that the stimulus is transmitted 
along the sensory fibre to some part of the central nervous system in 
which there is a path of communication between the receptive sensory 
cells and the emissive motor cells, and then from the motor cells along 
the motor nerve-fibres to the muscle or muscles involved. This is spoken 
of as a reflex arc, and it is considered that whenever its continuity is 
broken the reflex necessarily ceases to occur. 

Theoretically a sharp distinction is drawn between reflexes and 
muscular contractions which result from direct stimulation of the mus- 
cle involved, or of its motor-nerve supply, but in clinical work this 
distinction cannot always be maintained, and there are not wanting in- 
vestigators who contend that many of the reflexes upon which most 
dependence is placed in diagnosis, including, for example, the knee- 
jerk, are the result rather of direct irritation of the muscle, either by 
sudden elongation or by some other method, than of the completion of 
a reflex arc by the primary stimulation of the peripheries of the sensory 
nerves. It must, of course, be understood that the integrity of the nerve 
supply of the muscle by which its tone is maintained is essential to the 
production of the normal contraction. The distinction between the two 
theories is more philosophical than practical. Muscular contractions 
can, of course, also be produced by the direct irritation of motor nerves, 



KEFLEXES 911 

either at their termination or in the course of the nerve-trunk, as, for 
example, the contraction of the little and ring-fingers which occurs 
when the ulnar nerve is forcibly pinched. It is customary to speak of 
a reflex whenever, as a result of mechanical stimulation, particularly 
in the form of percussion, or stroking, a motor response occurs. The 
number of reflexes is already considerable, and every year three or four 
additions are made, most of them unimportant. They are classified 
in various ways : sometimes anatomically, as reflexes of the head, trunk, 
or extremities; sometimes according to the part to which the sensory 
stimulation is applied, as the skin, periosteum, bone, and tendon re- 
flexes ; sometimes into the skin or " superficial" and the " deep" re- 
flexes. 

The mechanism of the reflex action has been, ever since the first 
description of the knee reflex by Erb and Westphal, a matter of pains- 
taking investigation, and a considerable number of theories have been 
evolved. Taking the knee-jerk as the type of the deep reflexes the fol- 
lowing facts appear to have been definitely ascertained : 

The reflex is best elicited when the muscle is moderately stretched. 
It varies in general as the muscle tone; but this rule is subject to 
many exceptions, in states of hypotonia exaggerated reflexes being 
present (Muskens). It is modified by various external conditions, 
thus, attention to the reflex diminishes it; distraction of the patient's 
attention by either psychical or motor occupation increases it. It is 
usually increased as a result of fatigue or cachexia. Any diminution 
of the functional activity of the sensory fibres leading to the lumbar 
portion of the spinal cord diminishes or abolishes the reflex. Complete 
destruction of the motor fibres leading from the lumbar portion of the 
cord to the quadriceps extensor also abolishes the reflex. Partial de- 
struction of the peripheral motor neurones may be compatible with the 
persistence of the reflex, and even some degree of exaggeration, as in 
amyotrophic lateral sclerosis. Destruction of the centre for the reflex in 
the lumbar cord causes abolition of the reflex. Complete destruction of a 
segment or segments of the spinal cord above this centre usually causes 
loss of the reflex, but occasionally it persists, and is even exaggerated. 
If the posterior columns of the spinal cord are destroyed, as, for exam- 
ple, in tabes dorsalis, it is abolished. If the lateral pyramidal columns 
are destroyed it is greatly increased. Lesions in the brain involving 
the motor centres commonly cause a great exaggeration. Lesions not 
involving the motor centres usually cause a moderate exaggeration, but 
the reflex may be lost if the lesion is situated in the frontal lobes or in 
the cerebellum. 



912 THE EYE AND NERVOUS SYSTEM 

It appears from an analysis of these facts that the mechanism of 
the reflex involves more than a sensory and a motor fibre and the con- 
necting link in the spinal cord. Therefore it has been customary to 
assume the existence of a second arc, the afferent fibres being the con- 
tinuation upward of the sensory fibres passing from the periphery to 
the cord, and the efferent fibres being the axis cylinders of the upper 
motor neurones, the arc being completed by some of the commissural 
fibres of the brain. This second arc appears to have a double function ; 
that is to say, the persistence of the sensory part is necessary to the 
persistence of the reflex ; the loss of the motor portion tends to enhance 
the reflex. It must be remembered, however, that the destruction of 
the sensory portion some distance above the entrance of the nerve fibres 
into the cord occasionally may occur without loss of the reflexes, and 
if this injury to the sensory portion occurs above the nuclei of the 
columns of Goll and Burdach in the medulla, the loss rarely occurs. 

It is of course difficult to understand how the two limbs of an arc 
could exhibit so much difference in action. It may be assumed, for 
the sake of argument, that the upper arc is not really an arc at all; 
that the motor fibres passing from the cortex are concerned — with ref- 
erence only to reflex action — merely with the control of muscle tone, 
and particularly with the inhibition of involuntary movements (Drei- 
faser schema). Therefore, if for any reason, this inhibitory influence 
is removed, the reflex, of necessity, becomes exaggerated. It is more 
difficult to understand why the destruction of the sensory columns 
should diminish or abolish the reflex. It is supposed, for example, that 
this destruction always involves the peripheral sensory nerves that lead 
to the lower reflex arc. But this does not accord with the observation 
so frequently made that it can now be regarded as thoroughly estab- 
lished, that complete or even partial transverse lesion of the spinal cord 
in the upper dorsal or lower cervical portion is usually but not invaria- 
bly associated with loss of the reflexes, which is not due merely to loss 
of sensation. Numerous explanations have been suggested, according 
to which this loss is not due so much to the destruction of the sensory 
columns above the point of lesion as it is to a degeneration of the 
peripheral motor neurones and of the cells of Clarke's column, which 
has been found in the segments in which the reflex arc is completed, or 
to an oedema of the lower part of the spinal cord inhibiting its func- 
tional activity, or to loss of certain influences emanating from the 
cerebrum or the cerebellum, or to disturbance of the circulation in the 
lower part of the cord leading to degeneration of the ganglion cells, or 
to the effect of shock upon the lower part of the cord, although the 



EEFLEXES 913 

reflexes in the part above the lesion are not affected; or to degenera- 
tion of the posterior lumbar roots, this is called tertiary degeneration 
(Brasch) ; or to hypotonia. Van Gehnchten suggests that there may 
be increased inhibitory action. Aside from the degeneration of the cells 
in Clarke's column, no histological evidence has been discovered of in- 
jury to the sensory neurones. There appears to be some relation be- 
tween the amount of injury clone to the cord and the duration of the 
inhibition of the reflexes, at least in dogs. This loss is associated with 
a highly developed nervous system, for, according to Sherrington, in 
the dog and cat transverse sections of the cord cause exaggeration of 
the knee-jerks, and sometimes in human beings and in the ape the loss is 
not permanent. 

The way in which the sensory stimulation occurs has been the sub- 
ject of some dispute. In all the cutaneous reflexes it appears to be 
due to the stimulation of the cutaneous sensory nerves, whether that 
stimulation is perceived in consciousness or not. In the periosteal 
reflexes it is supposed that the sensory nerves in the periosteum receive 
and transmit the impulse. In the case of the simple muscular con- 
tractions due to direct irritation of the substance of the muscle, or its 
fascia, reflex action probably does not occur, or plays a subordinate role, 
and the same is true of muscular contractions due to irritation of a 
nerve trunk such as occurs in Chvostek's phenomenon. The dispute, 
however, has referred particularly to the tendon reflexes. The reflex 
occurs because a sharp blow is struck upon the moderately stretched 
superficial tendon of the muscle. Tendons are so poorly supplied with 
nerves, and particularly sensory nerves, that it does not seem pos- 
sible that the reflex is a result of direct irritation. Therefore, it has 
been surmised that it is due to the irritation of the overlying skin, the 
underlying periosteum (both of which hypotheses may be dismissed, 
particularly the former, as the reflexes occur in conditions of anaesthesia 
of the thigh or if the tendon is exposed) ; or to stimulation of the sen- 
sory nerves in the muscle by vibrations communicated to it, or by its 
simple sudden elongation. There can be no doubt, with reference to 
the knee-jerk, at least, that a distinct, powerful sensory impression 
occurs, sufficient — when the inhibitory influence of the motor fibres is 
removed — to cause contraction not only of the muscles affected by 
the blow upon the patellar tendon, unimuscular, but also of other 
groups of muscles in the same leg, multimuscular or even in the oppo- 
site leg. ^nen this occurs it is spoken of as a crossed reflex. From 
the fact that degeneration of the posterior columns of the cord inhibits 
the reflex, it seems not unlikely that the fibres of muscular sense are 
58 



914 THE EYE AKD NEKVOUS SYSTEM 

the ones chiefly concerned in the production of the so-called tendon 
reflexes. But the impossibility of separating these fibres in the nerve 
trunks renders all views upon this subject entirely hypothetical. 

The essential nature of reflex action is not clearly understood. Re- 
flexes were originally supposed to be involuntary movements of escape. 
Thus, the drawing up of the leg when the sole of the foot is tickled 
is apparently an attempt to remove the part from the source of the 
irritation. But it is impossible to explain all reflexes in this manner. 
It has been supposed that reflex action was the muscular response to 
a local irritation, psychology indicating that in the vast majority of 
cases an external stimulus precedes muscular activity, and that the 
psychical stimulus which may occasionally replace this represents 
merely stored-up external sensory impressions. According to this view, 
all action is essentially reflex in nature, but some of the reflexes are 
comparatively simple and quite regular, whilst others are complex, 
irregular, and apparently purposeful. Although this view may be 
entirely satisfactory to the psychologist, it is of no particular advantage 
to the clinician. He is compelled to regard certain activities produced 
by sensory stimuli as reflexes upon whose existence or alteration diag- 
noses may be based. All other forms of movement, if of clinical sig- 
nificance, must be considered from a totally different standpoint. It 
is therefore from a clinician that we have received the most ingenious 
and, in some respects, most satisfactory explanation. Von Striimpell 
believes that these reflex movements are vestiges of the complex co- 
ordinated reflexes of the lower animals, which in human beings, as a result 
of the increase in the cerebral motor activities at the expense of the 
spinal motor activities, have been reduced to insignificant and appar- 
ently purposeless movements. Therefore, he believes that when the 
cerebral controlling influence is removed, the reflexes become exag- 
gerated. As a matter of fact, the reflex interval is shorter in apes 
and idiots than in normal persons (Silbertini). Of course it is neces- 
sary to omit from consideration all those movements due either to 
direct irritation of the muscles or to direct irritation of the motor nerves, 
which are not properly reflexes, but merely manifestations of the in- 
herent and essential contractility of muscular substance. 

Unfortunately, there is still considerable dispute regarding the 
division in which many of the reflexes should be classified ; some hold- 
ing that they are due merely to direct irritation of the muscle or its 
tendon; others that they are true reflexes following the sensory nerve 
to the central nervous system, and returning along the course of a motor 
nerve. It is not impossible that in a few instances the reflexes may be 



EEFLEXES 915 

mixed, or at least in health may be merely muscular contractions, but 
when the inhibitory influence of the central motor mechanism is re- 
moved become true reflex movements. 

In studying reflexes for the purpose of utilizing the knowledge 
obtained for diagnosis or prognosis, certain factors must be considered: 
(1) The condition of the patient at the time the reflex is tested; this 
involves not merely the physical, but also the mental condition, states 
of excitement, fright, or preoccupation often having considerable in- 
fluence upon reflex activity. (2) The temperature of the room. (3) 
The mechanism employed for eliciting the reflex. (4) The position of 
the patient. With regard to the reflex itself, it should be noted (1) 
whether it occurs promptly or slowly ; ( 2 ) whether the response is nor- 
mal, exaggerated, or diminished; (3) whether other muscles than 
those usually concerned are involved in the response, and (4) the effect 
of the investigation upon the patient. 

In general it may be said that conditions causing increased myo- 
tatic irritability increase reflex activity. Mental emotion, such as 
fright, often inhibits reflex action. Great excitement, on the other 
hand, may enhance it. Chilliness increases it, and extreme cold, suffi- 
cient to numb the peripheral nerves, appears either to diminish or 
abolish it. In sleep it is diminished or even absent (Kosenbach). 

Nearly all reflexes may be elicited by the hand, but a great variety 
of instruments have been devised for the purpose of demonstrating them 
more readily. The most generally useful is the rubber percussion 
hammer which has a triangular piece of firm white rubber for its head, 
the blow being struck with the apex of the triangle. The ordinary per- 
cussion hammer may also be employed. Instruments have also been 
devised particularly for measuring the knee-jerk, so that the blows 
struck upon the tendon shall be uniform in force. It may be said in 
reference to all these instruments that up to the present time no clinical 
method for measuring reflex action has been devised that has such a 
degree of accuracy as to be in the least useful, and it is still necessary 
to estimate all reflexes according to the scale of " diminished, normal, 
or exaggerated," the determination to which one of these three classes 
any given reflex belongs depending entirely upon the judgment of the 
physician. Doubtless many errors are made, but on the whole the 
results have been of great service. It is possible that the method of 
Pandy for measuring the force of the knee-jerk may be of clinical 
value for the detection of marked paresis. 

For the purpose of description, the reflexes will here be considered 
according to a convenient rather than a logical classification. The re- 



916 THE EYE AKD KEKVOUS SYSTEM 

flexes of the head, upper and lower extremities, and of the trunk will 
be grouped into tendon, cutaneous, and muscle reflexes. No attempt 
will he made to treat exhaustively those forms which are of doubtful 
nature and little or no clinical significance. 

REFLEXES OF THE HEAD. 

There are no tendon reflexes in the head. The other forms of reflex 
action, aside from the pupillary reflexes, that may be elicited are : 

The Supraorbital Reflex. — This reflex was described by McCarthy 
in 1900 and consists of a slight, brief twitching of the orbicularis pal- 
pebrarum, most pronounced in the outer half, when a blow is struck 
upon the trunk of the supraorbital nerve. The reflex disappears in 
cases of destructive injury to the supraorbital nerve, causing anaesthesia 
in its area of distribution, and in peripheral facial paralysis. In cases 
of injury to the central nervous system no characteristic alteration 
usually occurs. A crossed supraorbital reflex may develop (Sailer). 
This may occur either under normal conditions, in which the twitching 
of the eye on the opposite side is very slight, or in cases of peripheral 
facial paralysis, in which it is quite distinct. Occasionally other move- 
ments occur, such as twitching of the ala of the nose or lifting of the 
corner of the mouth. These phenomena, however, are much more com- 
mon in connection with the malar reflex. It has been claimed that the 
supraorbital reflex is not a true reflex, but is due to percussion upon 
the fibres of the orbicularis palpebrarum that spread upward from the 
eyebrow (von Bechterew). The crossed reflex is explained by assuming 
that the fibres on the opposite side may interlace. It seems difficult, 
however, to account for the disappearance of the reflex in cases in which 
the supraorbital nerve has been cut or injured (McCarthy). The chief 
clinical significance of this reflex appears to be that when it is present it 
indicates that the supraorbital nerve on that side is intact, and that there 
is no peripheral or facial palsy. When it is absent it indicates that 
either the supraorbital or facial nerve has ceased for some reason to 
convey impulses. As facial paralysis is usually easily detected, when 
it is absent the lesion can be localized to the supraorbital nerve. Of 
course, when present it may be uncertain whether the supraorbital nerve 
is affected or not. Under these circumstances, the existence of the 
crossed reflex would serve to indicate that the supraorbital nerve upon 
the paralyzed side is intact. 

Chvostek's phenomenon consists of a sudden twitching of the muscles 
of the corner of the mouth, ala of the nose, and the external portion of 
the palpebral fissure when the trunk of the facial nerve is struck just 






REFLEXES 917 

after it winds around the posterior ramus of the lower jaw. This is 
not a true reflex, but simply exalted susceptibility of the nerve trunk 
to external stimuli. In certain cases this may be so great that per- 
cussion merely upon one of the branches of the nerve will suffice to 
produce the muscular reaction. Occasionally simple jDercussion upon 
the malar bone or pressure upon the eyeball (Fuchs) produces the same 
effect. This reflex is strictly pathological, and, as far as we know, 
pathognomonic of tetany, in all forms of which it may occur. Its 
absence does not exclude the existence of tetany, particularly in those 
forms grouped together as non-idiopathic ; that is, associated with vari- 
ous other conditions, such as cachexia thyreopriva, pregnancy, dilatation 
of the stomach, etc. It is, however, regarded as of such importance 
that when it is absent it is usual to speak of the " incomplete type." 

The malar reflex is either a muscle or a periosteal reflex, my opinion 
inclining strongly to the former view. In normal conditions percussion 
over the malar bone may cause a slight twitching of the upper angle 
of the mouth on the same side, but ordinarily produces no muscular 
response. In cases of recent facial paralysis of peripheral origin the 
myotatic irritability of the degenerating muscles is exalted, and per- 
cussion at this point causes contraction of the elevator of the upper 
angle of the mouth and movements of the ala of the nose. If the degen- 
eration — as is usually the case — is at all pronounced, the movements 
will be exceedingly sluggish, the angle of the mouth moving up slowly, 
and slowly resuming its previous position. In facial palsy of central 
origin the malar reflex occurs more frequently than in health, and the 
movements are much quicker. Clinically, the presence of the malar 
reflex in peripheral facial paralysis merely indicates that the muscle 
is not wholly degenerated, a fact which can, of course, be ascertained 
by the electric current. 

The chin-jerk is produced by tapping downward upon the teeth when 
the lower jaw is relaxed and partially open. For this purpose either 
a small flat object, such as tongue-depressor, may be rested upon the 
lower teeth, or the finger placed over the mental prominence. If the 
reflex occurs there is a sharp upward movement of the lower jaw. It 
appears doubtful whether this is a reflex. It is unquestionably exag- 
gerated in cases of myotatic irritation, such as cachexia, and 1 have 
obtained it in deep ether anesthesia. It is also likely to be increased 
in functional nervous conditions, such as hysteria with exaggeration 
of the reflexes. It does not appear to be altered in any characteristic 
way as a result of cerebral lesions, and bilateral peripheral lesions of 
the motor nerves that would be involved (facial, trigeminal) are so 



918 THE EYE AKD KEKVOUS SYSTEM 

rare that there are no recorded observations regarding their influence 
upon it. It may, therefore, be regarded as of no clinical significance. 
It may also be elicited by tapping upon the insertion of the masseter 
(von Bechterew). 

Other reflexes of the head involve particularly irritations arising 
from the 'mucous membrane in the nose and throat. These include the 
various nasal and pharyngeal and palatal reflexes. Their discussion, 
however, does not come within the scope of this article. Von Solder 
has described a lateral movement of the jaw when the cornea is irri- 
tated. Pressure upon the eyeball through the eyelid may produce the 
same effect (Fuchs). 

REFLEXES OF THE UPPER EXTREMITY. 
The bicipital reflex is a tendon reflex that is obtained by striking 
upon the radial tendon of the biceps, producing a contraction of the 
muscle and slight lifting of the upper arm. The reflex is best elicited 
in the following manner. The forearm is flexed upon the arm at little 
more than a right angle. The arm is then grasped just above the elbow 
in such a manner that the thumb rests upon the biceps tendon. This 
is then slightly compressed and a blow struck upon the dorsum of the 
thumb. It is often convenient to allow the arm of the subject to rest 
upon that of the examiner. The biceps is a true tendon reflex. Its arc 
appears to consist of the sensory nerve-fibres passing from the muscle 
to the fourth and fifth cervical segments of the spinal cord, and then 
downward along the brachial plexus and the musculo-cutaneous nerve. 
The reflex is not invariably present in health. It is usually exaggerated 
in functional nervous conditions associated with a general increase in 
the reflex activity, and is almost invariably increased in lesions of the 
brain involving the motor mechanism of the arm. It is lost in lesions 
of the posterior columns of the cervical cord, as in late tabes dorsalis 
or in the cervical form of this disease; possibly even more constantly 
than the patellar reflex. It is also lost in cases of destruction of the 
peripheral nerves, such as injury to the brachial plexus. It diminishes 
in proportion to the wasting of the muscle in the various forms of myop- 
athy that involve the muscles of the arms. Owing to its occasional 
absence in perfectly healthy individuals, inability to obtain it is not 
of great clinical significance unless it is unilateral. Exaggeration 
of this reflex, if associated with paresis or paralysis of the arm, may 
be regarded as a definite sign of injury to the motor portion of the cere- 
bral hemisphere of the opposite side. Bicipital and tricipital clonus 
are very rare. 



EEFLEXES 919 

The tricipital reflex consists of contraction of the triceps muscle when 
its tendon is struck a sharp blow. It is best elicited by bending the 
forearm upon the arm at about a right angle and then striking just 
above the prominence of the olecranon. If the reflex is present there 
will be a sharp contraction of the muscle and extension of the arm. 
Care must be taken not to strike upon the muscle. This reflex is in all 
respects closely analogous to the biceps and is subject to the same modi- 
fications by various lesions. It occurs in about two-thirds of all healthy 
persons. 

The supinator reflex is a contraction of the supinator muscle when 
a blow is struck upon the tendon as it passes over the radial bone. It 
is best elicited by having the forearm flexed upon the arm and at rest. 
There is usually slight flexion and supination of the arm. There is 
some doubt as to whether this is really a tendon and not a periosteal 
reflex. It is absent in about one-eighth of all healthy persons, is likely 
to be exaggerated in functional nervous disease, and is occasionally 
exaggerated on the paralyzed side in cerebral hemiplegia. It is of little 
clinical significance. 

Striking upon the extensor or flexor muscles of the forearm causes 
them to contract, giving rise, respectively, to extension and flexion of the 
fingers. This is not a reflex, but merely an expression of muscular irri- 
tability, the reaction occurring most distinctly in states of cachexia, and 
in conditions in which the muscles are easily stimulated mechanically. 

The hypothenar reflex consists of a contraction of the abductor of 
the little finger when pressure is made over the pisiform bone or when 
the end of the little finger is pinched. The contraction is usually tonic 
in character and is indicated by the appearance of a groove on the ulnar 
side of the hand. Occasionally there are a series of light twitches in 
the tonically contracted muscle. It is not constant, and nothing defi- 
nite is known regarding the conditions that cause it to be increased — 
if such a change could possibly be recognized — or to be lost. I have 
observed it in conditions of anaesthesia of the hand, such as unilateral 
anaesthesia from hysteria, and in anaesthesia associated with hemiplegia 
of cerebral origin. 

In some cases a 'palmar reflex may be obtained. 

REFLEXES OF THE LOWER EXTREMITY. 
Tendon Reflexes. — The knee-jerk is a contraction of the quadriceps 
tendon in response to a blow struck upon the patellar tendon, and was 
first described by Erb and Westphal. Clinically it is the most impor- 
tant of all the reflexes, and, as one of the first known, has been most 



920 THE EYE AND NEKVOUS SYSTEM 

extensively studied. It may be elicited in a variety of ways. If the 
patient is lying on the back the hand should be placed under the knee 
and lifted until the angle formed by the thigh and leg is about 120°. 
A sharp blow is then struck upon the patellar tendon either with the 
finger, the side of the hand, or a percussion hammer. There is a sudden 
contraction of the quadriceps which causes the patella to move upward, 
and, if sufficiently vigorous, raises the foot slightly from the bed as a re- 
sult of a forward kick. If the patient is sitting in a chair one thigh may 
be thrown over the other and the leg allowed to hang loosely. A blow 
is then struck as before and almost always results in a slight forward 
kick. If the patient sits on the edge of a table and allows the legs to 
swing the reflex can often be obtained very readily. In certain cases 
of extreme muscular atrophy, as a result of emaciation, the tendon may 
be so loose that the reflex cannot be obtained in any of the ordinary 
positions. Under these circumstances, if the leg is flexed strongly 
upon the thigh, it may be possible to stretch the tendon sufficiently 
to obtain a slight muscular contraction when it is struck. If the reflex 
is so diminished that it cannot be obtained by any of these procedures 
it may be re-enforced according to the method of Jendrassik. This 
depends essentially upon the fact that anything in the form of mus- 
cular or intellectual effort increases the vigor of the reflex. It is there- 
fore customary to give the patient some simple task to perform, such, 
for example, as an attempt to pull apart the hands with the fingers 
locked together. At the same time he should be instructed to look away 
from the knee. Sometimes it is sufficient to give him a simple calcu- 
lation in mental arithmetic. A convenient modification is to have him 
grasp a dynamometer or the physician's left hand ; the physician counts, 
" one, two, three," the grasp and the blow being performed as nearly as 
possible at the same moment. 

After the stimulus is applied there is perceptible interval before the 
muscular contraction occurs, which is called the latent period or reflex 
interval. It has been measured by numerous observers working with 
a great variety of instruments and in normal persons is probably about 
0.03 second. It varies considerably, however, in different individuals, 
and in the same individual under different circumstances. It is short- 
ened by conditions that increase reflex activity; and by mental condi- 
tions that diminish inhibition, microcephaly, mania and epilepsy. 
The duration of the contraction is also variable (0.1-0.5 second). It 
is greatly increased in spastic conditions. The curve of the muscular 
contracture has also been studied, but has yielded no information of 
importance regarding reflexes. 



KEFLEXES 921 

In testing the knee-jerk it is not sufficient merely to watch the 
excursion of the leg. Xot infrequently, if the muscles are weak, no 
movement in the leg is produced, and yet the reflex may be actually 
exaggerated, as is shown not merely by the violent contraction of the 
muscle, but also by the involvement of other groups of muscles in the 
thigh. It is therefore always advisable to rest one hand upon the mid- 
dle of the upper surface of the thigh. Occasionally the reflex can be 
detected by placing the finger upon the tendon and striking a blow upon 
the finger, the twitching produced by the muscle being then readily 
perceived. Instruments have been constructed for measuring the knee- 
jerk. They consist of an arc having approximately the radius of the 
length of the leg, with an indicator that is thrust forward by the kick 
of the foot. As it has been explained that the kick is not propor- 
tionate to the activity of the reflex, it will be obvious that none of 
them is of any value. At most they may serve to indicate an increased 
degree in the activity of the reflex in the same individual, and there- 
fore may be useful in experimental work to determine the effect of 
various influences. It is always advisable, but rarely convenient, to test 
the knee-jerk with the thighs bare. Very frequently there is slight 
twitching of the adductors on the same side, and occasionally of the 
adductors or of the quadriceps on the opposite side. This is nearly 
always true if there is much exaggeration of the reflex. In estimating 
the reflex we have no standard of measurement, and are accustomed to 
use the terms " normal," " diminished," " absent," "increased," and 
" greatly increased" for the purpose of indicating roughly its degree. 
Unfortunately, the degree of variation is considerable in different indi- 
viduals, and even at different times in the same individual. The fol- 
lowing points may be of some value in the estimation. 

The reflex is absent if, when the muscles are completely relaxed, 
and the patient is intelligent enough to employ the method of Jendras- 
sik, and the physician is certain that the blow is struck over the patellar 
tendon, — that is, between the lower edge of the patella and the tubercle 
of the tibia, — no contraction of the muscles takes place. It is desirable 
that the leg should be carefully inspected, and at the same time that 
the free hand should be laid over the muscles of the thigh in order to 
perceive a contraction that might be invisible. 

The reflex is certainly diminished if under ordinary conditions it 
cannot be obtained, and the response is slight when re-enforcement is 
employed. A visible response in a subject who has not previously 
been tested cannot be regarded as an indication of any pathological 
process. 



922 THE EYE AND NERVOUS SYSTEM 

The knee-jerk is certainly exaggerated if a slight tap upon the tendon 
causes the leg to fly out in an almost straight line; frequently, under 
these circumstances, two or more distinct twitches may be felt in the 
quadriceps muscle; if other muscles of the thigh, or of the opposite 
thigh, are involved; if there is patellar clonus; if the latent period 
is shortened and the duration of the muscular contraction lengthened, 
or if it is elicited by tapping on the periosteum of the tibia. 

The knee-jerk is said to occur in all normal individuals. There is 
no doubt, however, that the limits of variation are very wide. In some 
persons apparently normal there are times w T hen it can only be obtained 
by re-enforcement. In others it is invariably abnormally active. It is sub- 
ject to great variation in disease. It is usually lost in cases of disease 
of the posterior columns, as, for example, in tabes and Friedreich's 
ataxia. In tabes it is absent on both sides in about TO per cent, of all 
cases, and in about 15 per cent, more is pathologically altered. It may, 
however, even when lost, reappear in the later stages of the disease. 
It is lost in cases of total destruction of the peripheral nerves. It 
is reduced proportionately to the degeneration of the muscles in the 
various forms of myopathy. It is lost in cases of degeneration of the 
ganglion cells in the anterior cornua of the cord that control the quad- 
riceps muscle. In partial injury of the peripheral nerves, such as occurs 
in multiple neuritis, it is nearly always absent or greatly diminished; 
but in a few instances, whose nature is not clearly understood, it may 
be exaggerated. It is somewhat variable, also, in total transverse lesion 
of the cord. In the majority of recorded cases it has been entirely 
abolished by total transverse lesions in the dorsal, or even in the lower 
cervical, region, but a few cases have been recorded in which it persisted 
and was even exaggerated. Reference has already been made to this 
subject. There is no constant change in incomplete transverse lesion 
above the lumbar enlargement. 

The knee-jerks are always exaggerated if there is degeneration of 
the central motor neurones without degeneration of other parts of the 
spinal cord; and there may be actual clonus of the quadriceps muscle 
when the patellar tendon is struck, giving rise to a series of kicks of the 
leg, and these kicks, or at last the first one or two, may be so violent that 
the leg is extended ad maximum. Contractures of the adductors and 
occasionally of the quadriceps of the other leg are also nearly always 
present. Clonus of the patella usually co-exists and may be of a perma- 
nent character, — that is, continue as long as the pressure is maintained. 
This degeneration of the lateral columns occurs in the doubtful primary 
lateral sclerosis, and in cases of various cerebral lesions involving •the 



EEFLEXES 923 

motor tract. It occurs in the apparently idiopathic degenerative disease 
of the cord known as amyotrophic lateral sclerosis. In syringomyelia or 
in partial lesions of the dorsal or cervical cord arising from pressure as 
a result of disease of or of injury to the spinal column, the knee-jerk is 
usually increased, the increase ranging from a moderate exaggeration to 
(rarely) the extreme spastic type. In nearly all these cases there are 
associated sensory disturbances which aid in rendering the diagnosis 
clear. These may be paresthesia, often in the form of a girdle sensation 
at the level of the lesion, dissociation of sensation, as a rule more pro- 
nounced at first in the foot, but often ascending; a partial or complete 
anaesthesia, the latter sometimes involving all forms of sensation and 
limited by a horizontal line extending around the trunk at the site of the 
lesion. A distinction may be drawn between the exaggeration of the 
patellar reflexes with and without paralysis. In cases of hemiplegia the 
knee-jerk is usually greatly increased on the paralyzed side, in 92 per 
cent, of the cases, and moderately increased on the sound side, the 
contractility of the muscle being not in the least affected by the absence 
of voluntary innervation. In the first few days the reflex may be 
diminished or lost. Striimpell has observed a diminution in old hemi- 
plegias. In the syndrome to which the name primary spastic paraplegia 
was formerly given, the power of the muscles may be only slightly im- 
paired, but their extreme spasticity renders it difficult for the -patient 
to control them. In lesions of the upper portion of the cord the paralysis 
may be complete and yet the reflex be exaggerated. In functional ner- 
vous conditions, such as hysteria and neurasthenia, the knee-jerks are 
nearly always increased ; although very rarely they may be absent and 
hypotonia be present. There is no clonus and the patellar jerk, as a 
rule, does not show nearly the same degree of exaggeration. The power 
and voluntary control of the muscles in these cases may be intact. In 
general diseases of the brain the knee-jerks are variably affected. In 
cases of disease of the cerebellum or frontal region they may be in- 
creased or decreased, and periods of increase and decrease may alternate 
in the same case. In cases of disease of the cerebral hemispheres asso- 
ciated with increased intracranial pressure, as, for example, tumor, 
hydrocephalus, or meningitis, the knee-jerks, as a rule, are increased. 
In cases of brain tumor, careful statistics indicate that they may be of 
some aid in local diagnosis, although their behavior is not constant. 
Miiller, in fifty-five cases of tumor of the frontal lobes, found the 
patellar tendon reflex absent eleven times, normal nineteen times, and 
increased twelve times; in two cases it was lost on the side opposite 
to that of the tumor. Williamson found it absent in 20 per cent, of 



924 THE EYE AND NERVOUS SYSTEM 

forty-five cases of lesion of the frontal lobes. It is apparently absent in 
about 3 per cent, of tumors in other parts of the cerebrum. Reh finds it 
diminished in 34 per cent, of all cases of brain tumors. In tumors of 
the cerebellum it is lost in 41 per cent, of the cases according to Voss, 
and in 29 per cent, of the cases, according to Dinkelburg. In a case 
that I observed it disappeared and reappeared twice. The abdominal 
reflex is usually preserved, but this appears to be of no value as a dif- 
ferential sign. In chronic encephalitis — that is, in general paresis — 
the knee-jerks are usually increased, but occasionally are diminished or 
absent, especially after flaccid apoplectic attacks. The cases in which 
this occurs are usually recorded as the tabetic form, and lesions of the 
posterior columns of the cord are found at autopsy. Sometimes they are 
unequal. In insanity there is no characteristic change in the knee-jerk. 
In epilepsy the knee-jerks are, as a rule, increased, particularly in those 
congenital epileptics whose brains show actual lesions. In shock they 
are usually lost, and for this reason are often absent immediately after 
an epileptic attack. In uraemia they may be increased (Fiirstner). In 
certain general diseases the knee-jerks may be altered. In diabetes they 
are usually lost ; in febrile conditions, especially typhoid and tuber- 
culosis, they are increased. In croupous pneumonia it has been asserted 
and denied that they are diminished. When two conditions co-exist that 
affect the reflex in opposite ways, the result is variable. It has been 
observed to appear in the paralyzed limb in hemiplegia occurring in the 
course of tabes (Westphal). It is now generally admitted that the arc 
is completed in the third and fourth lumbar segments, with occasional 
variation to a segment above or below. There have not been wanting 
investigators, however, who believe that the arc is completed in seg- 
ments at higher levels, or even in the brain, but little importance can be 
attached to their views. According to Sherrington, the sensory nerve 
in apes is the branch leading from the vastus internus muscle to the lum- 
bar plexus. After section of all other sensory nerves, excepting this, the 
reflex can still be produced. The motor nerves are, of course, those 
passing to the different members of the quadriceps and adductor muscles 
on either side. 

The Patellar Reflex. — This consists of a sharp contraction of the 
quadriceps muscle drawing up the patella when that bone is forced 
downward by a blow. In other words, it is a reaction of the quadriceps 
muscle to sudden extension. The reflex is best elicited if the leg is 
extended. The patella may either be grasped between the thumb and 
index-finger and pushed forcibly down, or the finger may be laid trans- 
versely across the upper border and struck a smart blow with the per- 



KEFLEXES 925 

cussion-hammer ; or some round object, such as a lead-pencil, may replace 
the finger, or the ingenious instrument devised by Mills, which consists 
essentially of a ring encircling the patella, the lower end provided with 
a handle and the upper end with a slight enlargement upon which the 
blow is struck. The patellar jerk is not nearly so constant as the knee- 
jerk. It occurs, however, in the majority of normal individuals. In 
cases of spasticity of the muscles of the thigh the jerk is violent and 
may be repeated in the form of a tonic spasm, known as patellar clonus. 
This reflex has the same significance as the knee-jerk, but may be tested 
with advantage in patients who, through disease, stupidity, or ignor- 
ance of the language, cannot or will not relax the leg sufficiently to 
obtain a satisfactory test of the knee-jerk. As clonus indicates disease 
of the pyramidal columns, it is of considerable clinical importance. 

The adductor reflex consists of a sudden contraction of the adductor 
muscles on the inner side of the thigh when a blow is struck just above 
the internal condyle of the femur. There is usually a slight adduction 
of the thigh and occasionally a slight painful sensation. The reflex is 
best obtained if the thigh is flexed at about a right angle upon the trunk 
and the knee flexed upon the thigh. It is not invariably present in 
health, about 34 per cent., but is always present in spastic conditions, 
or, in fact, in any condition that gives rise to exaggeration of the knee- 
jerk. Reflexes may also be occasionally obtained by striking upon the 
hamstring tendons. Eor this purpose the patient should lie upon the 
face, the leg should be lifted from the bed by an assistant until the 
tendons of the biceps or of the semimembranosus and semitendinosus 
are relaxed, then the tendon to be tested should be moderately com- 
pressed by the thumb upon the dorsum of which a sharp blow is struck 
with a hammer. Eor some reason these reflexes have never excited the 
interest of clinicians and are rarely tested. < It is not known whether 
they are present in all normal persons, but they appear to be subject to 
the same conditions of increase and decrease as are the other tendon 
reflexes of the leg. 

The Achilles-tendon reflex consists of a sharp contraction of the mus- 
cles of the calf if a blow is struck upon the Achilles tendon in a state 
of moderate tension. To elicit this reflex the leg should be flexed upon 
the thigh at about right angles; the foot is then forcibly thrust upward 
until the tendon is tense, when it is struck a sharp blow. There is a 
contraction of the soleus and more or less extension of the foot. It 
seems to be generally admitted now that this reflex is produced only 
by the soleus muscle, and, in fact, is more easily obtained if the gastroc- 
nemius is relaxed. Both the afferent and the efferent fibres pass through 



926 THE EYE AND NERVOUS SYSTEM 

the trunk of the sciatic nerve. The arc is completed in the fifth lumbar 
and first sacral segments. The reflex is very constant, occurring, accord- 
ing to Bramwell and Kollarits, in all healthy persons under fifty years, 
and according to Strassburger in 07 per cent, of healthy persons. It 
disappears in disease of the posterior columns or of the posterior roots 
of the lumbar and sacral regions of the cord, sometimes, indeed, before 
the knee-jerk. Therefore, its loss, even if only unilateral, may be an 
early sign of tabes, or even general paresis. It is also lost in disease of 
the cord which contains the arc. It is often absent in sciatica, in which 
disease the knee-jerk usually persists, and when absent may indicate 
an actual neuritis. Under these circumstances the prognosis is graver. 
It is usually lost in complete transverse lesion of the spinal cord above 
the level of the arc. Occasionally when it persists and the knee-jerk is 
lost, or if it is lost and the knee-jerk persists, in cases of traumatism or 
focal disease of the lower portion of the cord, its study may contribute 
materially to correct localization. It is exaggerated in all conditions 
that give rise to exaggeration of the knee-jerk. 

Ankle-clonus is a manifestation of the Achilles-tendon reflex pro- 
duced by a slightly different technique. The leg is moderately flexed 
upon the thigh, the muscles completely relaxed, and the foot then thrust 
forcibly upward and the pressure maintained. Normally, after the 
sudden extension of the muscle there is a sharp contraction, perhaps 
followed by a slighter one. In certain functional neuroses or in states 
of fatigue there may even be a series of contractions diminishing rapidly 
in force. If there is actual degeneration of the superior motor neurones 
there will be a rhythmical to-and-fro movement of the foot which will 
persist as long as the pressure is continued, sometimes decreasing, some- 
times increasing in rapidity and in force, this variation occasionally 
being quite rhythmical in character. Ankle-clonus is an almost certain 
sign of lesions of the central nervous system ; indeed, it has been seri- 
ously doubted by many competent authorities (Mills) whether or not 
it occurs in functional nervous disease, although there appears to be 
no doubt that in rare instances it does so occur ; but the very fact that 
these instances are so uncommon leads unavoidably to the suspicion 
that temporary structural lesions may exist. In rare conditions it may 
be present when the knee-jerk is absent. A form of ankle-clonus 
may be produced in perfectly healthy persons if, when sitting at such 
a height that the foot rests easily upon the ground, it is lifted upon 
the toe and a series of voluntary oscillations is made. After a longer 
or shorter time the oscillations become involuntary, and there is a true 
clonic spasm that usually ceases spontaneously in a short time. In 



KEFLEXES 927 

states of fatigue, cold, or exhaustion from prolonged sickness it may 
merely be necessary to rest the ball of the foot upon the ground. It 
is important to differentiate between true ankle-clonus and this form 
which occurs in states of impaired muscular activity. 

The Skin Reflexes. — These are of considerable importance. 

The plantar reflex is produced by tickling the sole of the foot. There 
is a sudden withdrawal upward of the leg as a result of flexion at the 
hip and knee, dorsal flexion at the ankle, and plantar flexion of the toes. 
It therefore simulates a co-ordinated movement of escape, involuntary 
in character, produced, apparently, as well if the patient is unconscious 
as if conscious. Not infrequently only the toes move. The afferent 
and efferent fibres pass through the sciatic nerve, and the reflex arc is 
completed in the second and third sacral segments, although, on account 
of the involvement of the psoas and the muscles below it, the motor 
centres in the lumbar and lowest dorsal segments must also be involved. 
This reflex is subject to great variations in normal persons. In some it 
is apparently absent, and others are able voluntarily to prevent its 
occurrence. The thickness of the sole of the foot appears to be of slight 
influence. In functional nervous diseases, particularly in hysteria and 
neurasthenia, it is usually increased, and may even be crossed — that is, 
occur in both feet at the same time. Occasionally, in hysterical states, 
tickling of the sole of one foot will cause a general convulsive movement 
of the body, even although the patient may not perceive any tickling 
sensation. Organic disease of the central nervous system usually causes 
a moderate increase in the reflex which, under these circumstances, is 
limited to the leg to which the sensory stimulation is applied. Not in- 
frequently, however, the reflex is entirely lost in hemiplegia. It is 
invariably lost in disease of the peripheral sensory nerves of the legs. 

The Babinski phenomenon was first described in 1897. According to 
Schoenborn it is the only reflex of clinical value discovered since 1875. 
Babinski observed that, when there was disease of the pyramidal col- 
umns, instead of plantar flexion of the toes when the sole was irritated 
there was a slow dorsal upward movement of the great toe, sometimes 
accompanied, sometimes not accompanied, by the other toes. Numer- 
ous investigations have only served to confirm the importance and value 
of this reflex. Its absence does not, however, exclude pyramidal disease. 
It is usually easily elicited by stroking longitudinally on the sole of 
the foot with some blunt object, sometimes on the inner, but appar- 
ently preferably upon the outer side. Occasionally the reaction is vio- 
lent, and then the stroke should be very delicate. Sometimes it is 
necessary to use a hard or fairly sharp instrument, and to pass it along 



928 THE EYE AKD NERVOUS SYSTEM 

the more sensitive inner portion of the sole with some force. There 
is some dispute as to whether a sudden dorsal flexion of the great toe 
constitutes the Bahinski reflex or not. As it appears that even this 
sudden movement occurs very rarely, if at all, in normal persons or in 
those suffering from functional nervous disease, it may he accepted that 
it is really a pathological manifestation. Although usually associated 
with exaggeration of the tendon reflexes, this is not invariably the case. 
The value of the Babinski reflex compared with other signs of disease 
of the pyramidal tracts in the cord has been a subject of considerable 
investigation. It appears to be more constant than ankle-clonus, and 
as constant as the increase of the knee-jerk. It is said not to occur if 
the thalami have been injured, but this lacks confirmation. In my ex- 
perience, moreover, it appears to occur earlier and in certain cases to 
persist after the other signs have disappeared. In two cases of valvular 
heart disease which developed hemiplegia whilst under observation, 
probably embolic in nature, the Babinski reflex appeared during the 
first six hours, and before marked exaggeration of the other reflexes 
was apparent. In a case of hemiplegia with complete hemiansesthesia 
and abolition of all the tendon reflexes throughout the course of the 
disease, which lasted four months and terminated fatally, the Babinski 
reflex was occasionally obtained. In three cases of old hemiplegia with 
almost complete restoration of function and only slight increase in the 
knee-jerk and an imperfect clonus, the Babinski reflex was invariably 
obtained on the affected side. It has been claimed that it may occur 
in functional nervous diseases, especially hysteria, epilepsy, deep sleep, 
coma, and narcosis, and in typhoid fever. Miinch-Petersen, whose work 
requires confirmation, claims to have obtained it in a considerable pro- 
portion of healthy adults. Cohn regards it as of little value. It often 
occurs in normal infants, and therefore is of no clinical value before 
the age of two years (Morse). It has been questioned whether a perma- 
nent dorsal . extension of the great toe, such as sometimes occurs in 
Friedreich's ataxia or old hemiplegia, may not be a form of the Babin- 
ski phenomenon. Its arc is completed in the lower sacral region. 

Modifications of the Babinski Phenomenon. — The discovery of the - 
Babinski reflex stimulated great interest in the cutaneous reflexes in 
general, and particularly in those of the lower extremity. Oppenheim 
has obtained dorsal flexion of the toes in pyramidal disease by stroking 
the inner side of the leg, and his assistants have endeavored — although 
unsuccessfully — to give this the rank of an independent reflex. 
Schaefer has obtained the same reaction by pinching the Achilles ten- 
don. Hirschberg produces slight adduction of the foot somewhat sim- 



KEFLEXES 929 

ilar to the Babinski sign, by stroking the inner side of the instep. He 
claims that this reflex never occurs unless there is organic disease of the 
pyramidal columns, but his statement has been denied by Crocq. Men- 
del and von Bechterew have described a somewhat analogous reflex ob- 
tained by striking the dorsum of the foot. Under normal conditions 
there is dorsal flexion of the second to the fifth toes. In pyramidal dis- 
ease plantar flexion of the same toes may occasionally occur. 

The femoral reflex was described by Bemak. It consists of a plantar 
flexion of the toes and extension of the foot and leg if the upper portion 
of the anterior surface of the thigh is irritated. It only occurs in severe 
lesions of the cord, such as transverse myelitis occurring above the eighth 
dorsal segment, the arc, according to Bemak, being completed in the 
lower portion of the dorsal cord. It seems not unlikely, however, in 
view of the muscles involved, that the segments containing the arc are 
really lower than this. 

Unclassified Reflexes. — Certain phenomena whose exact nature has 
not been determined, but which are akin to reflex action, may some- 
times be observed. 

Kernig's sign was first described in 1888 in that birthplace of 
many reflexes, St. Petersburg. It consists of a spasmodic contraction of 
the biceps, semimembranosus, and semitenclinosus when, if the thigh is 
flexed at right angles to the trunk, an attempt is made to straighten the 
leg upon the thigh. There is some dispute as to the point at which Ker- 
nig's sign is said to occur, Kernig himself stating that the reflex was 
present if the leg could not be extended over an angle of 135° with the 
thigh. In its most typical form, however, the contraction takes place 
as soon as the angle of 90° is reached. This reflex may be elicited 
either by havim '"z patient sit in a chair and then attempting to 
pull forward the leg, or, if h: is lying on his back, flexing the thigh upon 
the trunk at right angles, and then attempting to extend the leg. One 
hand should be placed in the popliteal space and the sudden tension of 
the hamstring tendons is a very characteristic phenomenon. Kernig 
originally considered the sign pathognomonic of meningitis, in practi- 
cally every form of which he supposed it occurred, and subsequent in- 
vestigations apparently confirmed this opinion (Ems, Xetter, Herrick). 
Of late, however, it has been found to occur in focal brain disease 
(Sailer) and in the course of certain infectious fevers (Shields), and 
it does not occur in all cases of meningitis, and in particular it is likely 
to be absent in tubercular meningitis (Dieulafoy). It is, however, still 
a valuable sign because, although not pathognomonic, it occurs in more 
than 90 per cent, of all cases of acute meningitis, and very rarely in 
59 



930 THE EYE AND NEKVOUS SYSTEM 

other conditions. Therefore, if found, the probabilities are strongly in 
favor of the existence of meningitis. 

A reflex whose nature is not clearly understood is Sinkler's toe- jerk. 
It consists of forcible flexion at the hip and leg when the great toe is 
flexed strongly on the sole. It appears to occur in states of extreme 
spasticity, and I have been able to elicit it several times in cases of 
transverse myelitis or spastic paraplegia. Nothing appears to be known 
concerning its mechanism, but the arc must be completed in the lumbar 
portion of the cord, and it is probably one of those vestigial reflexes, 
only developing when inhibition is entirely removed, of Avhich Strum- 
pell speaks. The excitation appears to be due to traction upon the 
extensor muscles of the foot. 

Front-tap was first described by Gowers. It is elicited as follows : 
The leg is flexed slightly dorsally by the observer, and a blow is struck 
upon the tibialis anticus muscle. In about 40 per cent, of normal per- 
sons there is plantar flexion of the toes. The reflex appears to be 
more common in various forms of functional nervous disease, hysteria, 
neurasthenia, etc., and to be absent invariably in hypotonic conditions, 
such as tabes dorsalis. 

The tibialis phenomenon (Striimpell) consists of the contraction of 
the tibialis anticus muscle when the thigh is drawn towards the body. 
As the patient lies upon the back, one hand is placed over the knee and 
the other over the dorsum of the foot. The knee is then lifted by the 
patient when, if the reflex is present, the tense tendon of the tibialis 
anticus muscle is felt by the hand over the foot. This reflex occurs in 
cases of spastic hemiparesis, and may be of some practical value. 

REFLEXES OF THE TRUNK. 

Apparently there are no tendon-reflexes connected with the trunk. 
Two important skin-reflexes have been described, the abdominal and the 
cremasteric. The abdominal reflex consists of a sharp contraction of 
the muscles on one side of the abdominal wall when a light stroke is 
made from that side, passing obliquely downward and outward. This 
reflex is produced by irritation of the cutaneous terminations of the 
intercostal nerves that continue downward from the intercostal spaces 
and are distributed to the skin of the abdomen. The afferent and effer- 
ent fibres pass through the intercostal nerve trunks, and the reflex is 
therefore completed in the dorsal region of the spinal cord. It is 
customary to recognize two parts : the reflex of the upper and of the 
lower quadrants, or the upper and lower abdominal reflexes. There is 
no actual anatomical or physiological dividing line. This reflex is 



KEFLEXES 931 

present in 98 per cent, of normal persons, but varies very considerably 
in different persons or in the same person at different times. It is in- 
creased by the conditions that increase the skin reflexes ; it is abolished 
by lesions of the spinal cord which destroy the dorsal region. Some- 
times if only one part and not the other is involved it serves, in a sense, 
as an aid to localization. It is often active in tabes dorsalis, and may 
be present in cases of hysterical anaesthesia, but is lost in cases of 
organic anaesthesia of the abdomen. The hypogastric reflex, recently 
described by von Bechterew, consists of a deepening of the suprain- 
guinal region when the skin of the inner upper part of the thigh is irri- 
tated. According to Crocq it does not occur exclusively in women as 
was at first believed. 

The cremasteric reflex consists in the upward jerking of the testicle 
when the skin on the inner surface of the thigh is irritated. It is most 
readily elicited by pinching, scraping, or pricking the skin over the 
adductor muscles about one inch below Poupart's ligament. The up- 
ward movement of the testicle is sudden and independent of any con- 
traction of the scrotum, but may be associated with twitching of the 
abdominal muscles. The contractility of the cremasteric muscle varies 
extraordinarily in different individuals. A small proportion — esti- 
mated at 1 or 2 per cent. — have voluntary control over the muscle 
and can elevate the testicle at will, and in these cases the reflex is pro- 
nounced to the degree of exaggeration. In other individuals without 
obvious lesion of the central or peripheral nervous systems the reflex 
may be persistently absent. In other cases it may be extremely varia- 
ble. Under pathological conditions, it is increased in functional nervous 
states; it is usually increased in conditions giving rise to increased 
spasticity, although it may be diminished in hemiplegia on the affected 
side, and is lost in conditions causing hypotonicity, as, for example, 
tabes dorsalis. It is said to be exaggerated in sciatica. It is a reflex 
of moderate clinical importance. The contraction of the dartos muscle 
lining the wall of the scrotum is occasionally mistaken for the cremas- 
teric reflex. It is, however, independent and differs distinctly in char- 
acter, being a slow contraction coining on at an appreciable interval 
after the skin has been irritated. It persists for a short time, and then 
relaxation ensues. This reflex appears to be independent of nearly 
everything excepting complete organic anaesthesia of the part irritated. 
It is of no clinical importance. 

Von Bechterew has described a pyramidalis reflex, a slight contrac- 
tion in the lower part of the abdomen just above the symphysis when 
the skin above this region is irritated. 



932 THE EYE AND NEEVOUS SYSTEM 

The periosteal and muscle reflexes are not important. 

Scapulohumeral Reflexes. — Von Bechterew has described a series of 
reflexes connected with the scapula, which are best elicited by striking 
on the spinal edge just below the superior internal angle. This causes 
a slight contraction of the infraspinatus and occasionally of the supra- 
spinatus muscle, and adduction and external rotation of the humerus. 
In cases of hemiplegia in which the muscles are spastic the contractions 
may be more extensive, involving also the trapezius and deltoid, and 
causing, therefore, abduction instead of adduction of the arm. Not 
infrequently I have observed a contraction of the muscles of the oppo- 
site shoulder. It would seem, therefore, that the scapular reflex must 
be a true reflex and not merely the result of muscular irritation. There 
is no doubt, however, that striking upon the bellies of the spinati mus- 
cles gives rise to contraction of these muscles with results that simulate 
the reflex under ordinary conditions. Steinhausen regards it as a 
periosteal reflex constant in normal persons. 

Iliac Reflexes. — Striking upon the crest of the ilium gives rise to 
various contractures of the muscles attached to the pelvis. These re- 
flexes are increased by all conditions giving rise to increase of the mus- 
cular irritability. They have received very little attention from neu- 
rologists, and little is known excepting the fact of their existence. 

Negro has described as the gluteal reflex a sharp tetanic contrac- 
tion of the gluteal muscles when pressure is made over the sciatic 
foramen in cases of sciatica. It may assist the diagnosis in doubtful 
cases. 

Mention may be made of the idiopathic muscular contraction, or 
myoidema. This consists of a tonic spasm of isolated fibres of a muscle 
which have been subjected to vigorous mechanical irritation. It is best 
elicited in the pectoral muscles by tapping sharply upon them with the 
finger or hammer. This produces a small lump which rapidly dis- 
appears. It can also be elicited in the biceps by pinching it sharply, 
a ridge appearing along the line of the pinch. This reaction, although 
it can usually be obtained in normal persons who are thin and whose 
muscles therefore are not protected by a layer of fat, is most striking 
in cachectic conditions. It also is common in certain forms of insanity, 
as general paresis, dementia prsecox ; in epilepsy, cerebral syphilis, etc. 
It has no clinical significance, excepting as a confirmation of cachexia, 
which is, of course, readily recognizable by other means. 



EEFLEXES 933 



LITERATURE ON REFLEXES. 

Reflex action was first clearly described by Marshall Hall (Lectures on the 
Nervous System and its Diseases, Philadelphia, 1836, p. 33 et p.eq.) . His first com- 
munication was made in 1832 (see " Memoires of the Nervous System," London, 
1837). Among the earlier writers who observed some of the phenomena upon which 
the discovery of reflex action was based may be mentioned Whytt, 1751; Prochasky, 
1807; and Mtiller, 1833. The literature of this, as well as of other interesting 
discussions, during the early phases of neurology, is collected in a curious book, 
" Documents and Dates of Modern Discoveries in the Nervous System, London, 
1839." 

General. 

Goweks. Med.-Chir. Trans., vol. lxii. [Text-Book of Nervous Diseases. — Ed.] 

Sheeeington and Jendeassik. C. R. de la Sec. de Neurol., Thirteenth Internat. 
Cong., 1900 (quoted by von Kornilow) ; Philosoph. Trans. Royal Society of 
London, Series B, 1898, vol. cxc. p. 45; Neurolog. Centralbl., 1900, p. 979. 

Steenbeeg. Die Sehnenreflexe. [This book contains a bibliography almost com- 
plete to the time of its publication.] 

Steumpell. Neurolog. Centralbl., 1899, p. 617; Deutsche Zeits. f. Nervenheilk., 
vol. xx. p. 436. 

Mann. Monats. f. Psych, u. Neurol., 1898, 7, 410. 

Muskens. Neurol. Centralbl., 1899, p. 1074. 

Lugaeo. Riv. di Patolog. Nerv. e Ment., vol. iii.; Neurolog. Centralbl., 1899, p. 
264. 

Senatoe. Zeitschr. f. klin. Med., vol. xxxv. 

Beauee. Deutsche Zeitschr. f. Nervenheilk., vol. xviii. p. 284. 

Feaenkel. Deutsche Zeitschr. f. Nervenheilk., vol. xiii. 

Habel. Archiv. f. Psych., vol. xxix. p. 25. 

Mabinesco. La Semaine Med., 1898, vol. xviii. p. 153; Neurolog. Centralbl., 1898, 
p. 818, criticism by Bruns. 

Westphal. Archiv f. Psych., vol. xxx. p. 554. 

Geehaedt. Neurolog. Centralbl., 1898, p. 612. 

Van Gehuchten. Journal de Neurologie et de Hypnologie, 1897, June 26; 1898, 
June 5. 

Bischoff. Wiener klin. Wochenschrift, 1896. 

Keon. Deut. Zeitschr. f. Nervenheilk., vol. xxii. p. 24. 

Balint. Deutsche Zeitschr. f. Nervenheilk., vol. xxiii. p. 178. 

Beasch. Neurolog. Centralbl., 1899, p. 1115; Fortschritte der Med., vol. xviii. 

Sommee. Lehrb. der Psychopath. Undersuch., Berlin, 1899. 

Stewaet. Journal of Physiology, 1897, p. 61. 

Baetels. Neurolog. Centralbl., 1902. 

Tuenee. Journal of Nervous and Mental Disease, 1902, p. 321. 

Colliee. Brain, Spring, 1904. 

Ebb. Archiv f. Psych, u. Neurol., vol. v. p. 792. 

Westphal. Ibid., p. 803. 

Pandy. Neurolog. Centralbl., 1904, p. 449. 

Scheven. Neurolog. Centralbl., 1904, p. 488. 

Reflexes of the Head. 
Supraorbital Reflex. 
McCaethy. Neurolog. Centralbl., 1900. 
Von Bechteeew, Neurolog. Centralbl., 1900. 



934 THE EYE AND NERVOUS SYSTEM 

Hudovernig. Neurolog. Centralbl., 1901, p. 933. 
Sailer. Philadelphia Medical Journal, 1901. 
Lukacz. Neurolog. Centralbl., 1902. 

Malar Reflex. 
Von Bechterew. Neurolog. Centralbl., 1901, p. 931. 
Sailer. Philadelphia Medical Journal, 1901. 

Chin- Jerk. 
James. Scottish Medical Journal, Oct. 1900. 

Arm Reflexes. 
Mohr. Deutsche Zeits. f. Nervenheilk., vol. xix. p. 197. 
Fraenkel. Deutsche Zeits. f. Nervenheilk., 1900, p. 197; 1900, vol. xvii. 
Rennie. Australian Med. Gazette, March, 1900; Neurolog. Centralbl., 1901, p. 

450. 
Weisenburg. Journal of Nervous and Mental Disease, 1903, p. 681. 

Reflexes of Lower Extremity. 
Knee-Jerk. 
Erb. Arch. f. Psych, u. Neurologie, vol. v. p. 792. 
Westpiial. Ibid., p. 803. Neurolog. Centralbl., 1885, p. 412. 
Bernhardt. Die Periph. Nerven., NothnagePs System, vol. xi. 37. 
Brissaud. Recherches Anat. Pathologiques et Physiologiques sur la Contracture 

Permanente des Hemiplegiques, Paris, 1880. 
Eulenberg. Zeits. f. klin. Med., vol. lv. p. 179. 
Sternberg. Loc. cit., p. 31; Die Sehnenreflexe, p. 30. 

Kollarits. Zeits. f. Nervenheilk., vol. xxiii. p. 89. (1000 normal adults.) 
Blauch. Arch. f. Psych, u. Nervenheilk., vol. xii. 1882, p. 471. (700 children; 

absent five times.) 
Berger. Centralbl. f. Nervenheilk., 1879, No. 4 (found it absent in 1 per cent. 

of normal persons ) . 
Pelizaeus. Arch. f. Psych, u. Nervenheilk., vol. xiv. Heft 2. 
Spitzer. Zeits. f. Nervenheilk., vol. xix. p. 215. 
Shoenborn. Ibid., vol. xxi. p. 273. 
Brauer. Ibid., vol. xviii. p. 284. 
Balint. Ibid., vol. xix. p. 414. 
Strumpell. Ibid., vol. xvii. p. 268. 

Nonne. Deutsche Zeits. f. Nervenheilk., vol. xxiv. p. 474. 
Muller. Ibid., vol. xxii. p. 375. 
Voss. Ibid., vol. xxi. p. 43. 
Finkelburg. Ibid., vol. xxi. p. 296. 
Reh. Monats. f. Psych, u. Neurol., vol. xv. p. 182. 
Westphal. Charite Annalen, 1899. 

Tumpowski. Deutsche Zeit. f. Nervenheilk.. vol. x. 1897. 
Furstner. Neurolog. Centralbl., 1898, p. 607. 
De Montyel. Annales Med.-psycholog., 1897, March and April. 
Lugaro. Neurolog. Centralbl., 1899, p. 264 (abstracted). 
Adamkiewicz. Ibid., 1899, p. 338. 

Ganault. These de Paris, 1898; Neurolog. Centralbl., 1899, p. 367. 
Brunn. Neurolog. Centralbl., 1899, p. 518. 
Brauer. Munch. Med. Woch., 1899, No. 2. 






KEFLEXES 935 

Williamson. Glasgow Medical Journal, Nov. 1899. 

Libertini. Arch, de Med. exp. et d'Anatomie Pathologique, Sept., 1900. 

Dercum. Journal of Nervous and Mental Disease, 1898, p. 615. 

Pfatjndler. Munch. Med. Woch., 1902, p. 1211. 

Koeder. Deut. Archiv f. klin. Med., 1903. 

Pandy. Neurolog. Centralbl., 1904, p. 449. 

Collier. Brain, Spring, 1904. 

Patellar- Jerk. 
Gowers. Text-Book of Nervous Disease, 2nd Ed. 
Mills. Journal of Mental and Nervous Disease, 1899, March. 

Adductor Reflex. 
Remlinger. Revue de Med., 1901, p. 72. 

Achilles Tendon Reflex. 
Kollarits. Deutsche Zeits. f. Nervenheilk., vol. xxiii. p. 89. 
Strasburger. Ibid., vol. xvii. p. 306. 

Lembach. Ibid., vol. vii. p. 943 (in 92 per cent, of 400 cases of tabes). 
Babinski. Gaz. des Hopitaux, 1896; Bull, et mem. de la Soc. des Hopitaux de 

Paris, 1898, Oct. 21. 
Muskens. Neurolog. Centralbl., 1899, p. 1084. 
Ziehen. Deutsche Med. Woch., 1894, No. 33. 
Biro. Deutsche Zeits. f. Nervenheilk., vol. xix. p. 188. 
Knapp. Monats. f. Psych, und Neurol., vol. xvi. p. 327. 
Remlinger. Revue de Neurologie, 1901, p. 72. 
Mills. Journal of Nervous and Mental Disease, 1899, p. 131. 
Leri and Follet. Neurolog. Centralbl., 1901, p. 481. 
Biro. Ibid., 1901, p. 686. 

Plantar Reflex. 
Collier. Brain, Spring, 1899. 
Cohn. Neurolog. Centralbl., 1899, p. 582. 

Babinski Phenomenon. 
Babinski. Progres Medicale, 1898, p. 166; La Semaine Med., 1898. July 27. 
Van Gehuchten. Journal de Neurologie, 1898, April 5; Revue de Neurologie, 

1899. 
Crocq. Ibid., 1901. 

Langdon. Cincinnati Lancet-Clinic, Feb. 1900. 
Schoenborn. Zeits. f. Nervenheilk., vol. xxi. p. 273. 
Specht. Monats. f. Psych., vol. xix. p. 81. 
Pfeiffer. Monats. f. Psych., vol. xiv. p. 270. 

Koenig. Arch. f. Psych., vol. xxxiii. p. 311; Neurolog. Centralbl., 1899, p. 610. 
Passini. Wien. klin. Woch., 1900, No. 41. 
Schneider. Berl. klin. Woch.. 1901, p. 946. 
Bickel. Deutsche Zeits. f. Nervenheilk., vol. xxii. p. 24. 
Munch-Petersen. Deutsche Zeits. f. Nervenheilk., vol. xxii. p. 177. 
Giudiceandrea (quoted by von Kornilow). 
Vires and Calmettes. Revue de Neurologie, 1900. 
Von Kornilow. Zeits. f. Nervenheilk.. vol. xxiii. p. 216. 
Glorieux. Policlinique, 1898, p. 381. 
Cohn. Neurolog. Centralbl., 1899, p. 582. 



936 THE EYE AND KEKVOUS SYSTEM 

Schuelek. Ibid., 1899, p. 585. 

Kalischer. Virchow's Archiv, vol. civ. 

Collier. Brain, Spring, 1899. 

Leri. Neurolog. Centralbl., 1901, p. 141; Revue Neurolog., 1903, No. 14. 

Cronzon. Ibid., 1901, p. 142. 

Morse. Pediatrics, 1901, January. 

Walton and Paul. Journal of Nervous and Mental Disease, 1900, June. 

Cestan and Le Sourd. Gaz. des HSpitaux, 1899, Nov. 22, p. 1249. 

Buzzard. British Med. Jour., 1899, vol. i. p. 1077. 

Van Epps. Journal of Nervous and Mental Disease, vol. xxviii. No. 4. 

Tumpowski. Medycyna, 1901; Neurolog. Centralbl., 1901, p. 663. 

Chodzko. Gazeta Lekarska, 1901; Neurolog. Centralbl., 1901, p. 663. 

Schoenborn. Neurolog. Centralbl., 1901, p. 10. 

Homburger. Ibid., 1901, p. 698. 

Goluflam. Ibid., 1903, December 1. 

Barns. Review of Neurology and Psychiatry, 1904, May. 

Homberger. Neurolog. Centralbl., 1902. 

Bramwell. Review of Neurology and Psychiatry, 1903, June. 

Marinesco. Revue Neurologique, 1903, No. 10. 

Richter. Miinchener Med. Woch., 1903, No. 24. 






Modifications of the Ba'binski Phenomenon. 
Oppenheim. Monats. f. Psych, u. Neurol., 1902. 
Cassirer. Ibid., p. 37. 
Pfeiffer. Ibid., pp. 270, 585. 
Verger and Abadie. Progres Medicale, 1900, No. 17; Neurolog. Centralbl., 1900,, 

p. 859. 
Schaefer. Neurolog. Centralbl., 1899, p. 1016. 
Hirschberg. Revue Neurologique, 1903, No. 15. 
Mendel. Neurolog. Centralbl., 1904, p. 197. 
Von Bechterew. Neurolog. Centralbl., 1904, p. 609. 
Crocq. Jour, de Neurol ogie, 1901. 

Unclassified Reflexes. 
Kernig. Berlin Klin. Woch., 1884, p. 829. 
Friis. Copenhagen Thesis, 1877 (quoted by Netter; also Ugeskrift for Laeger, 

1892, pp. 407, 431). 
Netter. La Semaine Medicale, 1898, p. 281. 

Herrick. American Journal of Medical Sciences, 1899, vol. cxviii. p. 35. 
Sailer. Ibid., May, 1902. 
Shields. Ibid. 

Dieulafoy. Clinique Med. de l'Hotel de Dieu, 1898-1899, p. 347. 
Sinkler. Medical News, Dec. 1, 1888. 

Strumpell. Deutsche Zeits. f. Nervenheilk., vol. xx. p. 436. 
Roglet. Journal de Medecine, 1900, Oct. 10. 

Front -Tap. 
Walton and Paul. Journal of Nervous and Mental Disease, 1903, p. 341. 

The Abdominal Reflex. 
Schoenborn. Deutsche Zeits. f. Nervenheilk., vol. xxi. p. 273. 
Dinkler. Ibid., vol. xxii. p. 23. 
Ostankow. Neurolog. Centralbl., 1898, p. 140; 1900, p. 765 (abstract). 






GAITS 937 

The Cremaster Reflex. 
Steiler. Deutsche Zeits. f. Nervenheilk., vol. xxvi. p. 285. 
Geigel. Deutsche Med. Woch., 1892, p. 166. 
Schoenbokn. Deutsche Zeits. f. Nervenheilk., vol. xxi. p. 273. 
Ganault. These de Paris, 1898; Neurolog. Centralbl.. 1899, p. 367. 
Gibson. Edinburgh Medical Journal, 1901, p. 459. 

The Hypogastric Reflex. 
Von Bechterew. Neurolog. Centralbl., 1901, p. 647. 
Van Gehuchten. Journal de Neurologie, 1900. 
Von Kornilow. Zeits. f. Nervenheilk., vol. xxiii. p. 216 (calls attention to some 

early references to this reflex ) . 
Crocq. Journal de Neurologie, 1901. 

The Femoral Reflex. 
Remak. Neurolog. Centralbl., 1893, No. 15, 1900, p. 7. 

The Scapulohumeral Reflex. 
Von Bechterew. Neurolog. Centralbl., 1900, pp. 208, 1042. 
Haenel. Ibid., p. 399. 
Steinhausen. Ibid., 1901, p. 507. 
Pickett. Journal of Nervous and Mental Disease, 1901, No. 5. 

The Gluteal Reflex. 
Negro. Bull, del Policlin-gen. di Torino, II (Neurolog. Centralbl., 1898, p. 79). 

Myoidema. 
Bernstein. Neurolog. Centralbl., 1899, p. 1068. 

GAITS. 

THE GAIT AS A SYMPTOM OF NERVOUS DISEASE. 

Walking may be described as progression by placing one foot on 
the ground, supporting the body upon it, lifting the other foot to a 
slightly higher level, and swinging the leg forward ; this leg in turn 
becomes the support, and the first leg repeats the movements. The sup- 
porting leg is kept rigid by the contraction of the muscles, particularly 
those of the calf, the quadriceps, and the gluteal and psoas muscles, 
although practically all the other muscles of the limb are brought into 
play for the purpose of limiting movements at- the joints. During the 
act of walking there are two chief movements in the supporting limb: 
at the ankle-joint, in which the tibia rolls forward on the astragalus, 
and at the hip-joint, in which the thigh-bone rocks backward in the 
acetabulum as the body moves forward. There is also a slight rotary 
movement in the hip as the pelvis is swung around it in the effort of 
throwing forward the opposite leg. The knee-joint usually remains 
fixed until the movement forward becomes so great that the heel of the 



938 THE EYE AND NERVOUS SYSTEM 

supporting leg is lifted from the ground, when it begins to bend slightly. 
The shortening of the swinging leg is accomplished by tilting the pelvis 
and slight flexion at the thigh, knee, and ankle. The swing commences 
with a slight throw given with the toes as the foot leaves the ground, 
and terminates as the heel strikes the ground, when the forward incli- 
nation of the supporting leg becomes so great that the swinging leg no 
longer clears the surface. 

METHODS OF STUDY. 

The ordinary mode of walking may be modified by structural deform- 
ities, especially if in the skeleton, or by functional defects either in 
the muscles or in the nervous mechanism controlling them. These modi- 
fications have been studied usually by simple observation, but a variety 
of methods have been employed for the purpose of elucidating the final 
changes and making permanent records. The simplest method is to 
measure off a definite path on which the patient walks. The number 
of steps is counted and divided into the distance travelled. The length 
of each step is thus readily determined. By observing the patient care- 
fully from behind or in front, lateral deviations in the movements of 
the legs can be detected, and by placing the eye close to the ground it 
can be noted whether the feet are abnormally raised or not. This 
method, however, is scarcely better than direct observation (Weber). 

It is a distinct advance to employ some method by which the foot- 
prints themselves can be more or less permanently impressed upon the 
surface. This is, of course, done upon soft soil or sand, but the yielding 
character of the material produces certain alterations in the gait which 
render it slightly abnormal. Moreover, the preservation even of a single 
footprint by the method of making plaster casts is tedious and difficult. 

Vierordt was apparently the first to employ a color material, which 
was exuded by a specially prepared shoe. Gilles de la Tourette covered 
the sole of the foot with powdered oxide of iron, and then had the 
patient walk upon strips of rough paper, enough of the powder being 
caught in the interstices to leave a record. Still more complicated is 
the method of Monkenmoller and Kaplan, who employ an alcoholic 
solution of chloride of iron in which the stockings are soaked. The 
patient walks upon a piece of paper which is afterwards sprayed with 
an alcoholic ethereal solution of ammonium sulphocyanide, forming 
ferric sulphocyanide, and leaving a permanent dark red mark that can 
readily be photographed. The very thick wet stockings interfere slightly 
with the gait. 

I have used a simple modification which has proved so satisfactory 



GAITS 939 

that I believe I can commend it heartily. It consists in painting the 
feet with ordinary black paint, — that is, lamp-black dissolved in linseed 
oil. The paint should be somewhat thicker than that ordinarily supplied 
ready-made. It can best be smeared on the feet with a wad of cotton 
or gauze. Ordinary wrapping-paper is employed. This comes in rolls 
about four feet wide, from which strips of convenient length (usually 
from fifteen to twenty feet) can readily be cut. A single painting suf- 
fices for about twenty clear tracings from each foot, — that is, the patient 
can walk a much longer distance than the usual length of the strip of 
Avrapping-paper. The paint dries quickly and makes an impression that 
is practically permanent. I have records over four years old that are 
as clear as they were originally. The strips can be rolled up into very 
small compass, can easily be stored and classified, and photographic 
reproduction is very satisfactory. Jendrassik condemns these methods 
because, according to him, it gives us no more information than simple 
inspection. In this I think he is mistaken. Obviously it enables us 
to substitute for more or less erratic guessing actual measurements re- 
garding the length of the steps, the difference in the length of the steps 
in the two legs, the separation of the legs, and whether or not successive 
steps of the same leg are in a straight line. Moreover, the print of the 
foot can be carefully studied : whether the whole of the sole touches 
the ground, whether part of it remains permanently above the ground, 
and if any dragging occurs it is very distinctly shown. The great 
advantage of this method, however, is that it furnishes a permanent 
record infinitely preferable to any description that can be written. The 
chief objection to it is that no record is made of the movements of the 
feet while in the air. 

A very simple method which may be employed if no materials are 
at hand is to allow the patient to walk on the floor after the feet have 
been wet. Usually from five to ten steps with each foot will be recorded, 
and the water does not evaporate for several minutes. If the patient 
wears thick stockings which have been saturated with water a greater 
number of steps can be obtained. This will enable the observer to take 
satisfactory measurements of the foot-prints. 

The application of instantaneous photography to moving objects 
provided a new method for the study of the gait. To Muybridge, 
working at first independently and subsequently under the auspices of 
the University of Pennsylvania, the credit for the first complete study 
of the human gait is due. Not only can the individual phases of motion 
be studied by this method, but also by various methods the pictures can 
be thrown upon a screen in such rapid succession that the movement 



910 THE EYE AND NERVOUS SYSTEM 

appears to be reproduced. For scientific purposes it suffers under the 
disadvantage that pictures taken with the subject advancing directly 
towards or retreating from the camera are comparatively unsatisfactory. 
This method, with various changes, has also been employed by Marie, 
Dercum, Braune, Fischer, and Londe ; and Marinesco has used the 
more modern cinemetograph. 

Jendrassik has devised a modification which he considers superior 
to all other methods. It consists in having the patient walk in a dark 
room in front of a photographic plate and lens, and then by means of 
electricity producing momentary flashes of light at regular intervals. 
As a result the picture of the individual at various stages of the gait 
appears upon the plate. The objections to this method are numerous. 
In the first place, the subject, walks in a room alternately dark and 
bright, and as a result the gait must be to a certain extent disturbed. 
In the second place, and to my mind this is a more serious objection, 
the pictures upon the plate are superimposed ; it is very difficult, there- 
fore, to make a careful analysis. In the third place, it is practically 
impossible to take pictures with the patient walking toward or away 
from the camera. Nevertheless, this method, if carefully carried out, 
yields excellent results, and some of the pictures published by Jen- 
drassik leave little to be desired. 

Other methods that have been employed for attempting to record 
various movements graphically, either upon a rotating drum or by means 
of an apparatus squirting colored fluids upon a recording surface, are 
so cumbersome and unsatisfactory that they have been entirely dis- 
carded. 

The classification of the pathological varieties of gait is compara- 
tively simple theoretically. Practically, however, it is difficult, partly 
because of the great variation occurring in many of the forms, partly 
because of the not infrequent association of two or more disturbing 
factors. Broadly we may recognize two groups: those due to actual 
deformity of the skeleton and those due to disturbances in the neuro- 
muscular mechanism. The former, with the exception of a few forms 
of trophic disturbance of the bones, belong to the domain of surgery, 
and will not be considered in this article. The latter may be divided 
into five groups. First, flaccid paralytic lesions of the neuromuscular 
mechanism ; second, spastic paralytic lesions of the neuromuscular 
mechanism, each of these may be subdivided into the unilateral and 
bilateral forms, and they are often combined ; third, the group character- 
ized by loss of muscular co-ordination, — that is, the ataxic gaits ; and, 
fourth, a group clue to involuntary muscular movements, — the chorei- 



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I. II. III. IV. 

I. Case of Hemiplegia, Two Years Duration.— The impression of the riirht foot is weak ; there 
is some contraction of the muscles, causing accentuation of the arch. The alignment is good and 
there is very little dragging of the foot. 

II. Case of Hemiplegia of Long Duration.— The right foot i< turned out and drags distinctly 
(unfortunately the illustration has been retouched and does not show this). The steps are uneven and 
there is occasional staggering. 

III. Case of Cerebral Spastic Paraparesis.— The steps are short and uneven and there is a 
tendency to crossing the legs. 

IV. Case of Primary Neural Atrophy : that is, Paralytic Paraparesis.— The toes are turned 
out; the alignment is poor. The peculiar character of the footprint is due to the atrophy of the 
muscles of the sole of the foot. 



9 



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V. VI. VII. VIII. 

No. 5. The Gait of a Normal Adult Male.— The alignment is good ; the steps are equal ; the impres- 
sions are clear and distinct. The left foot is turned out slightly more than normal. 

No. 6. A Case of Tabes Dorsalis.— The alignment is poor; the distance between the steps is unequal; 
at one point the leg is thrown slightly to the side. The impressions vary in intensity, particularly with refer- 
ence to the arch of the left foot. 

No. 7. A Case Supposed to be Cerebellar Disease.— The type of gait is the late stage of ataxia with 
paresis. The steps are very short ; the legs are widely separated ; the alignment is poor. The steps are very 
unequal in length, and the impressions vary in intensity. It will be noted that the number of toes striking 
the ground varies in different impressions, showing a slightly spastic condition of the extensor muscles. 

No. 8. The Gait of a Normal adult Female. The steps are shorter, and the separation of the legs 
slightly greater than in the tracing taken from the adult male. The gait presents no abnormal features. 



GAITS 941 

form gaits. This comprises a number of very diverse conditions. 
Finally, there are several forms, different in themselves and not be- 
longing to any of the regular types. Before discussing these different 
classes I wish to mention the general features that should be most care- 
fully studied, because it is by means of the amount and degree of their 
deviation from the normal that the character of the gait is determined. 
First, the length of the steps ; whether the step made by one foot 
is longer or shorter than the step made by the other, — that is to say, 
whether the distance from the toe of the left foot to the heel of the right 
foot is more or less than the distance from the toe of the right foot to 
the heel of the left foot. Second, the degree of separation of the two 
feet; under normal circumstances, a straight line drawn between the 
footprints should touch or slightly cut the inner side of both heels. 
Third, the degree to which the toes are turned out; this is modified 
more or less by habit, but normally a straight line drawn through the 
centre of the heel passes through the ball of the great toe ; however, 
moderate deviations from this rule cannot be considered pathological. 
Fourth, the part of the foot which first touches the ground, whether the 
heel or toe ; this is also modified by habit, but it is excessively rare for 
any person normally to strike the ground with the ball of the foot first, 
although Ellis believes that certain savage races normally walk in this 
manner. Fifth, the way in which the foot leaves the ground ; whether 
it is thrown clear by the spring of the toes or whether they remain in 
contact with the surface for a longer or shorter time while the foot is 
dragged forward. Sixth, the way in which the sole of the foot rests 
upon the ground, in some cases the arch is distinctly lost, or the patient 
may walk upon the toes or the side of the foot. Seventh, the height to 
which the foot is lifted from the ground during the step. Eighth, the 
amount of lateral swing in the leg. Xinth, the manner in which the 
foot strikes the ground, whether forcibly or lightly. Tenth, the align- 
ment of the steps. In addition, such gross changes as cross-legged pro- 
gression or walking with crutches should be noted, and such finer 
changes as the behavior of the toes during the step. Under certain cir- 
cumstances, spastic dorsal flexion may prevent one or all of the toes 
from touching the ground at any point. 

THE PARALYTIC GAITS. 

The flaccid paralytic gaits are characterized by the absence of the 

reflexes in addition to the weakness of the limbs. As a general rule, 

these gaits occur in various forms of peripheral palsies and myopathies. 

If bilateral, such, for example, as occnrs in peripheral polyneuritis, the 



942 THE EYE AKD NERVOUS SYSTEM 

gait presents the following peculiarities: the steps are short, the feet 
are planted rather widely apart, and there may or may not be irregu- 
larity in alignment. The feet are lifted from the ground without 
swinging, the toe leaving last. The knee is lifted somewhat higher than 
normal from the ground, in order to permit the toe, which, as a result 
of toe-drop, is not raised, to clear the floor. The toe strikes the ground 
first and then the rest of the foot is planted upon the surface. During 
the step the foot remains flat upon the ground and does not aid in lift- 
ing the body. Walking is therefore accomplished chiefly by the muscles 
that move the thigh upon the pelvis. In the severer forms of this type 
of paralysis the weakness is so great that it is impossible for the patient 
to lift the feet from the ground, and they are dragged along, usually 
turning slightly outward. In these forms additional support is nearly 
always required, either by canes or crutches. As a rule, walking is 
attempted only in the milder forms. 

In anterior poliomyelitis, either unilateral or bilateral, the type of gait 
depends largely upon the particular muscles affected. If the involve- 
ment is extensive, walking without crutches is impossible. The legs 
are drawn forward and serve as a sort of third crutch to aid in pro- 
gression. Very often it is necessary to stiffen the joints by means of 
braces. If the peroneal muscles are involved there will be foot-drop, 
the leg will be lifted higher from the ground, and will be planted nearly 
flat, the toe touching first, but if the calf muscles are preserved there 
will be a distinct spring at the end of the step. In these cases the foot 
is usually turned out. If contractures have ensued, giving rise to 
club-foot, the condition becomes surgical rather than neurological, and 
the type of gait depends upon the nature of the deformity. 

In the ordinary forms of muscular dystrophy the ability to walk is 
preserved for a long time. The gait is quite characteristic. The steps 
are short, usually made with effort ; the legs are well separated so that 
the gait has a waddling character ; the feet are not lifted very well from 
the ground, and the knees are kept nearly completely extended. If at 
any time they become flexed the patient is likely to fall. The foot, 
however, usually clears the ground, and, unless the weakness is extreme, 
the alignment is good. As the muscles of the trunk are also frequently 
involved there may be considerable rocking of the pelvis which accentu- 
ates the waddling. 

In pseudomuscular hypertrophy the gait is essentially the same, the 
only difference being in the appearance of the limbs. In both forms 
the patient shows a peculiar method of arising from the floor which has 
been frequently described, but is so characteristic that it serves as an 



GAITS 943 

important symptom. He first assumes a sitting posture, then drawing 
the hody forward gets upon the hands and knees, then pushing the body 
backward he brings the hands close to the feet, grasps the legs, and 
gradually climbs up his own legs, supporting the trunk partly upon the 
arms and partly upon the legs. In the peroneal form of muscular dys- 
trophy and in some forms of polyneuritis the predominant characteris- 
tic of the gait is the extreme ataxia. This will be described in connec- 
tion with ataxic gaits. 

The Spastic Paralytic Gait. — Lateral Sclerosis. — Bilateral paretic 
disturbance of gait with spasticity of the muscles occurs as a result of 
cord or brain lesion. Pressure upon the cord above the lumbar enlarge- 
ment gives rise to a peculiar so-called spastic gait which was formerly 
believed to be due to sclerosis of the lateral columns. As a matter of 
fact, a descending sclerosis of these columns does occur, but very rarely 
as an idiopathic lesion. The steps are equal in length and may be short 
or long, according to the degree of the paralysis : the less the paralysis 
the longer the step. The feet are usually kept moderately close togther, 
often, indeed, there is a more or less pronounced tendency for the legs 
to cross. During the swing the leg rotates slightly outward to pass the 
other leg ; the leg is kept stiff and the foot extended. As a result, for 
a greater or less portion of the stride the toe remains in contact with the 
ground. The whole gait is of a peculiar stiff and awkward character. 
Although the toe touches the ground first, there is no spring to it, and 
the patient proceeds very much as if walking on stilts. It is charac- 
teristic of this condition that the toes of the shoes are very rapidly 
worn out, and that frequently the inside of the legs of the trousers, 
particularly at the knees, are also soon worn through. In forms in 
which the spasticity is the most pronounced condition the legs may 
even be crossed, giving rise to cross-legged progression. A modification 
of this form occurs in Little's disease. In this, however, there is marked 
foot-drop ; the patient therefore lifts the leg high from the ground in 
order to clear the toe : the so-called " chicken-gait." 

The unilateral spastic paretic gait is, in the vast majority of con- 
ditions, the result of cerebral lesion. The gaits, of hemiplegia are, as 
a rule, quite characteristic. The degree of modification, of course, de- 
pends largely upon the extent of the paralysis. In the mildest forms 
there is merely a slight limp. Careful observation and foot-print rec- 
ords, however, will show that even in this type the step on the hemiplegie 
side is somewhat shorter; that the toe drags for an instant after the 
step has commenced, and that, as a result of the stiffness of the leg, 
it is swung slightly to the side, the foot being lifted less than the leg 



944 THE EYE AKD NERVOUS SYSTEM 

on the sound side. The steps are usually in a straight line ; the foot 
is turned out, and not infrequently the feet are separated slightly 
more than in normal walking. The more severe forms are merely exag- 
gerations of these features, but of course present a very different appear- 
ance. The step is short, the feet are well separated, the toe clings to the 
ground for a considerable distance, sometimes throughout the entire 
period of the step, the leg is swung well outward, and usually the foot- 
steps on the affected side are no longer in a straight line, showing the 
existence of a greater or less amount of ataxia. As the muscles on the 
paralyzed side are not capable of tilting the pelvis, this is accomplished 
by throwing the body over to the opposite side, so that with each step 
it oscillates distinctly. The step on the sound side is usually character- 
ized by an exaggerated spring from the toe, and a marked hurrying of 
the swing so that as little time as possible is spent resting on the lame 
leg. If the condition becomes more severe the patient is obliged to 
support himself with a cane or crutch usually carried on the sound side, 
although if the arm has recovered sufficiently it is not uncommon for 
the patient to carry a crutch on the sound side and a cane in the other 
hand. 

GAITS DUE TO DISTURBANCE OF CO-ORDINATION. 

The ataxic gait is characterized by the following features : inequal- 
ity in the length of the steps ; defective alignment of successive steps ; 
irregular high lifting of the feet from the ground, and usually a some- 
what stamping type of progression, — that is, the feet strike the ground 
rather vigorously. As a result of the uncertainty and irregularity of 
the footsteps, the centre of equilibrium is continually disturbed, and 
the body is jerked forward and backward or to one side or the other in 
order to preserve the balance. 

In tabes dorsalis this type of gait occurs. All grades may be ob- 
served, from a slight impairment, which may be imperceptible unless 
special methods are used, to such a profound paresis and incoordination 
that the patient can only progress by the aid of two canes or crutches. 

The tracing of a tabetic gait is usually quite characteristic. The 
spaces between the successive footprints show a considerable amount of 
variation. Occasionally a footprint is seen far to one side, due to the 
effort of the patient to recover his balance. Even where the walking 
is more regular, it can be seen that sometimes the feet are slightly 
crossed, sometimes more widely separated than normally. The char- 
acter of the impression varies considerably. Sometimes the sole of the 
foot is pressed firmly against the ground ; sometimes the patient walks 



GAITS 945 

upon the inner and sometimes upon the outer side of the foot, In 
some impressions the arch is distinct ; in others it is faulty, apparently 
the result of irregular contractions of the muscles controlling it. The 
changes are always more pronounced the further the patient has walked. 
In the beginning the footsteps maintain a fairly good alignment, pos- 
sibly due to the greater concentration of the mind in the beginning of 
the effort. The toes are sometimes turned out; sometimes the foot is 
directed forward, showing irregularities in the rotation of the leg at 
the hip. Even slight variations in the intensity of the impression can 
be made out in most of the tracings. One of the most characteristic 
features of the ataxic gait is that, in spite of the obvious impairment 
of equilibrium, only occasionally is an effort made to compensate it 
by separating the legs and thus broadening the base. ^Moreover, a 
study of the tracings shows that, in spite of the apparently long stride 
due to the elevation of the foot from the ground, and the forcible way 
in which it is placed downward, the average distance between the steps 
is slightly less than in normal persons of the same size. 

The ataxic usually appears to walk hastily and heedlessly, but it 
will be observed that he keeps his eyes fixed carefully upon the ground, 
and, as far as he may, chooses his path. There is also a certain element 
of tenseness and rigidity in the whole poise that is merely an indication 
of the effort made. In nearly any form of ataxia a footprint tracing 
will exhibit the characteristic features, even although they are not per- 
ceptible to simple inspection. But in these cases the alteration may be 
made more obvious by employing certain modifications in the gait. The 
simplest of these is to have the patient walk with the eyes closed. Ordi- 
narily, as soon as the aid of vision is removed, the ataxia becomes much 
more pronounced. Walking backwards is accomplished with difficulty, 
and often results in falling. Walking upon an uneven surface is done 
very badly. For this reason such patients are very apt to fall over slight 
obstacles, particularly if entering a dimly lighted, unfamiliar room. 
They also walk badly in the twilight and at night. Indeed, it is in a 
measure a characteristic symptom of locomotor ataxia that the patients 
stagger in the early evening. Certain modifications occur in the gait 
as the result of the progress of the disease. Tabetic arthropathies pro- 
duce such a relaxation of the ligaments of the joint that it may bend 
freely in all directions. Under these circumstances, with each step the 
leg shortens as a result of bowing, either backward or laterally. The 
body therefore tilts to that side, and the other leg can only be swung 
or dragged a short distance forward until it is necessary to change the 
step. Walking becomes difficult, but the deformity may become extreme 

60 



946 THE EYE AND NERVOUS SYSTEM 

before it becomes impossible, even although both knees may be affected. 
Arthropathies may occur in the hip, but do not give rise to such char- 
acteristic alteration in the gait. 

In the advanced forms of tabes with marked paresis unaided loco- 
motion is impossible. The patient usually seeks the aid of one or, often, 
two canes ; the body is tilted forward, a good deal of the weight resting 
upon the arms, the steps are short, even dragging, and quite uncertain; 
the patient keeps his eyes fixed upon the ground as well for the purpose 
of placing the canes as of planting the feet. Even the movements of 
the arms are uncertain, and the canes are not carried forward in a 
straight line, but waver from side to side and are thrust against the 
ground in a manner that resembles the stamping of the feet. In cases 
of blindness as a result of optic atrophy in the course of the disease a 
curious modification takes place. The ataxia disappears almost entirely, 
and the patient may for a time be able to walk quite as well as any 
other blind person. As a rule, however, increasing paresis finally causes 
disability. As a result of careful education of the tabetic by means of 
systematic co-ordinated movements, gradually increasing in complexity 
(Fraenkel's method), so much improvement may ensue that even a 
severe ataxia may be converted into a mild form. In the tabetic form 
of general paresis the gait is modified in the same manner as in tabes 
dorsalis. 

The gait in Friedreich's ataxia is intermediate between the spinal and 
the pure cerebellar forms, although it resembles the spinal more nearly. 
As the disease progresses the inco-ordination becomes more and more 
severe until finally the patient is unable to walk at all. Not infrequently 
a deformity of the feet — a form of talipes equinus — adds a limp to the 
gait of inco-ordination. 

Cerebellar ataxia differs from spinal ataxia in several particulars. 
The co-ordination is usually more disturbed, therefore the steps are 
exceedingly irregular and lack alignment. Co-ordination is disturbed 
not only in the legs, but also in the trunk, and, as a result, there is 
considerably more swaying of the body, partly due to the inco-ordina- 
tion, partly to the violent efforts of the patient to maintain equilibrium. 
The gait is not made Avorse by closing the eyes, — that is to say, visual 
impressions aid the patient little if any. There may or may not be a 
marked sense of vertigo. If the cerebellar type is pronounced the patient 
frequently falls, and, of course, in the severer forms walking unaided 
is impossible. It is noteworthy that in the severe forms crutches or 
canes are not of as great assistance as they are in spinal ataxia, and 
the patient must be supported by one or even two attendants. 






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IX. x. XL 

No. 9. A Spastic Paretic Gait from a Case of Syringomyelia.— The paresis is in excess; the arch of 
the right foot is wholly lost, and that of the left foot is imperfect. The alignment is distinctly poor ; the length 
of the steps is unequal, and the toes are turned slightly out. It will be noted that the intensity of the impres- 
sions is unequal. 

No. 10. A Case of I. eft Hemiplegia of Six Months Standing.— The arch of the foot is wholly lost on 
the right side, and is imperfect on the leftside. These changes possibly antedate the lesion. The foot on the 
paralysed side is turned outward at an angle of 30 degrees. The feet are well separated; the alignment is 
poor : the steps are very short. There is no distinct dragging of the toes. 

No. 11. A Bilateral Spastic Gait.— The steps are short- the alignment is poor. The separation of the 
feet is unequal, but occasionally there is a tendency to crossing the legs. There is distinct dragging of the 
toes on both sides, the dragging of the foot being outward so that the knee can clear the knee upon the 
opposite side. 



GAITS 947 

Cerebral ataxia is not very common. It occurs occasionally as a result 
of focal lesion, and is usually on one side of the body. Under these 
circumstances, if slight, it may be detected in the footprint tracing by 
noting that on one side the steps are slightly unequal in length and the 
alignment is j30or. Bilateral ataxia may occur as a result of pressure 
from brain tumors or other conditions. It is usually of the cerebellar 
type. 

In multiple neuritis marked ataxia sometimes occurs constituting the 
pseudotabetic form. This is especially characteristic in the hypertro- 
phic form. 

Choreiform Gaits. — The gait of athetosis is characterized by irregu- 
lar movements not only of the legs, but also of the whole body. The 
muscles appear to relax and contract slowly, entirely independent of 
voluntary control, but at the same time a certain amount of control is 
possible, so that these irregular arhythmical movements can be partly 
inhibited and directed. By the exercise of great effort this inhibition 
and direction nearly always suffices to maintain the equilibrium. The 
patient walks with most irregular movements. The steps are unequal, 
the feet are planted irregularly, the body bends from side to side or 
forward and backward, the head is continually moving, and the patient 
appears as if falling backward or sidew T ays and to recover his balance 
almost by a miracle. Actual falling rarely occurs. This condition is 
not incompatible with a fair degree of intelligence. 

In ordinary chorea the gait is not typical. There is some jerkiness 
in the movements and occasionally paresis of the legs or of one side of 
the body, causing disturbances that resemble slightly those of hemi- 
plegia. 

THE GAITS OF FUNCTIONAL NERVOUS DISEASE. 

In hysteria the greatest variety of disturbance of gait may occur. 
All the organic forms may be simulated more or less perfectly, and, in 
addition, there are certain types that are peculiar to hysteria. The 
commonest is the so-called histrionic gait. This can best be described 
as a series of attitudes during progression, with steps of an exaggerated, 
but not particularly abnormal, character. The body is usually held at 
various angles, the legs are drawn forward with a sweeping motion, the 
feet are lifted high from the ground, and there is considerable oscilla- 
tion of the trunk. In these cases there is not really a true ataxia, 
although the footsteps may be somewhat uneven. In hysterical mani- 
festations the symptoms are usually more pronounced when the patienl 
knows he is under observation, but this is not invariably the case. 



948 THE EYE AND KEKVOUS SYSTEM 

Astasia-abasia is a somewhat more rare form which has been regarded 
as a separate disease. This is really the fear of falling. Only by the 
strongest urging can the patient be persuaded to attempt to walk at all. 
When he finally stands upright he clings to any object that is near him. 
If at last the patient is persuaded to walk, the steps are exceedingly 
short (not more than a few inches), and the patient appears to be in 
the utmost terror. If surrounding objects are removed he usually stands 
reaching out for support in an appealing fashion. He practically never 
falls, nor does he seem, as in the case of athetosis, to be continually 
upon the point of falling. In the majority of these cases there is usually 
a general anaesthesia which may account, in part, for the peculiarity of 
the gait. 

Although scarcely a disturbance of gait, certain other manifestations 
of hysteria have to do with the limbs. Particularly is this true of the 
dancing forms of pandemic hysteria, the jumper's disease, which is 
practically the same thing, and various other phenomena. The hysterical 
forms of ataxia, hemiplegia, spastic paralysis, etc., can be distinguished 
from the organic diseases, as a rule, without great difficulty. The move- 
ments are more irregular, exaggerated, and their character, in addition 
to the hysterical stigmata, such as disturbance of the color fields, anaes- 
thesia, tender points, etc., is so characteristic that a diagnosis can be 
made at a glance. 

In Thomsen's disease, or myotonia congenita, there is usually difficulty 
in starting to walk. The patient, if sitting down, will have to wait 
an appreciable instant before arising; if standing, there will also be 
an interval before the muscles relax sufficiently to allow the limbs to 
move. Ordinarily, in the milder forms, there is no further difficulty in 
progression after this preliminary stage is passed. The muscular spasm 
may, however, come on while the patient is standing, or even while 
walking, and then, owing to the inability of the muscles to balance the 
body, the patient frequently falls heavily to the ground. 

The gait of paralysis agitans is quite typical. It is described as the 
gait of festination, and it is usually stated that patients have difficulty 
in starting, then the steps increase in frequency and decrease in length 
until the patient is running along. In this condition he is unable to 
stop until he guides himself against some obstruction which he usually 
strikes, apparently with considerable force. In not all the cases of 
paralysis agitans, however, is the festinating gait typical. Very fre- 
quently patients have difficulty in starting to walk, but can then walk 
a considerable distance, the steps being made slowly, the distance be- 
tween the feet increasing, and the length of the steps decreasing. Even 



GAITS 949 

at this slow rate of progression voluntary stoppage is frequently im- 
possible. In other cases, instead of becoming shorter as walking con- 
tinues, the steps become distinctly longer, so that the patient is soon 
running with long strides. In taking footprint tracings of these cases 
I found one in which there was a marked dorsal extension of the toes 
when the sole of the foot was on the ground, so that the balls of the toes 
never came into contact with the ground, the patient touching with the 
heels and springing with the ball of the foot. As a matter of fact, the 
spring was insignificant, the foot being lifted and held almost horizontal 
to the surface. These patients also show retro- and propulsion, — that 
is to say, when standing or sitting they have a tendency from time to 
time to move or fall backward or forward. Patients with paralysis 
agitans, however, rarely fall, and, considering the difficulty in locomo- 
tion, it is remarkable with what readiness they undertake to walk either 
voluntarily or at the request of the observing jmysician. The nature 
of this gait has been the subject of some speculation, and it is supposed 
that the muscular rigidity characteristic of this disease does not enable 
the patient to recover the equilibrium after it has been slightly advanced 
in the course of movement. The station in these cases is quite typical. 
The patient bends slightly forward, the knees are slightly bent, the head 
slightly protruded, the neck is rigid, giving rise to a curious attitude, 
the elbows are thrown slightly back, the forearms are bent, and the hands 
slightly advanced. The whole attitude expresses an extreme degree of 
rigidity. 

The gait in sciatica is, as a rule, characteristic. The patient walks 
with the leg flexed slightly upon the thigh, giving rise to shortening and 
a distinct limp. The foot is usually kept nearly horizontal to the 
ground, and there is a little spring from the toe at the end of the step. 
The swing of the leg is shortened and the patient, in order to put as 
little weight as possible on the affected side, throws the trunk toward 
the other. He also stands in a somewhat characteristic attitude. The 
leg is slightly flexed, the heel sometimes a little drawn up from the 
ground, and the trunk inclined toward the sound side. 

DELAYED WALKING. 

The age at which a child learns to walk is of some significance. As 
a rule, a normal child should stand alone before the first year and should 
walk before eighteen months old. This, however, is subject to many 
exceptions in both directions. Many children, especially European 
children, who appear to develop somewhat earlier than American chil- 
dren, walk before they are a year old, and a child may be perfectly 



950 THE EYE AND NERVOUS SYSTEM 

normal in all other respects who does not walk until two years old, or 
even later. It is always advisable, however, to make a careful exami- 
nation of any child who does not walk at eighteen months. Interruption 
in the ability to walk is of great clinical significance. It is not un- 
common for children to walk a few steps at an early age, and then, as 
a result of fright from a fall, or of timidity, or of other unimportant 
causes, to refuse to attempt it again for several weeks. If, however, a 
child who has walked for any considerable time gradually loses the 
power, it usually means that some disease of the central nervous sys- 
tem is in progress. It is very common, for example, for children suffer- 
ing from the various forms of degenerative atrophy, particularly of the 
hereditary types, to lose the power of walking at about the time these 
diseases manifest themselves. Thus, in cases of the amaurotic form of 
family idiocy the children cease to walk about the age of two years. 
This is also true of many other forms of idiocy. 

REFERENCES. 

Vierordt. Das Gehen des Menschen, Tubingen, 1881. 

de la Tourette. Etude clinique sur la Marche, Paris, 1885. 

Marey. La photographie du mouvement, Paris, 1892. 

Blocq. Les Troubles de la Marche, Paris, 1892 

Braune and Fischer. Sachs. Abhandl., 1895-1900. 

Monkemoller and Kaplan. Neurolog. Centralbl., 1900, p. 798. 

Jendrassik. Deutsch. Archiv. fur klin. Med., vol. lxx, p. 81. 



CHAPTER XXIII. 

DEGEXERACY (DEGEXERATIOX, CONSTITUTIONAL 
DEFECTS, DEVIATION.) 

By G. L. WALTON, M.D. 

The terms degeneration and degeneracy in their academic sense 
denote a lowering in the scale of being, particularly with regard to 
moral qualities. The custom has crept in to apply them to all evi- 
dences, physical or mental, of deviation from the average normal, and 
to call the possessor of such evidences degenerate. The term superior 
degenerate, originally introduced to designate the individual with men- 
tal deviation, has been extended to include the possessor of minor signs 
of deviation, whether physical or mental. This use of language is 
unfortunate, since it implies a conclusion by no means established, and 
•conveys an opprobrium, lessened, it is true, by increasing familiarity, 
but in the majority of instances quite inappropriate. The desirability 
of introducing the purely descriptive terms, deviation and deviate, will 
be considered later. 

This subject, aside from its general importance, is of special interest 
to the neurologist, since the so-called stigmata of degeneration have to 
be constantly taken into consideration as bearing on the diagnosis and 
prognosis of nervous disorders. 

To the oculist the subject is of equal interest for two reasons. In 
the first place, the so-called stigmata of degeneration, a long list of 
which will be found at the end of the chapter, include a large number 
of ocular defects, such as astigmatism and other errors of refraction, 
difference in color of irides, flecks on the iris, coloboma, and albinism, 
besides such functional disturbances of vision as night blindness and 
achromatopsia. In the second place, it is of practical importance for 
the oculist, as well as for the neurologist, to recognize these signs, since 
they often indicate the constitution and character of the individual, 
whatever relation they may bear to degeneration. For in estimating 
the relation of eye-strain to headache or other nervous disturbances, it 
must be remembered that such symptoms may be constitutional as well 
as reflex. 

Among the most obvious and most uniformly listed " stigmata of 
degeneration," facial asymmetry conies first. A common form, but 

951 



952 THE EYE AND NEKVOUS SYSTEM 

unusual degree, of facial asymmetry is illustrated by Eigs. 1 , 2, and 
3. One eye appears higher than the other without corresponding ele- 
vation, or even with depression, of the mouth and chin. A tendency 
of this sort, apparent or real, will be noted in a large percentage of 
normal individuals without reference to righthandedness ; in a less 
number the eye, mouth, and chin will all appear higher on one side. A 
difference of one to three millimetres in the length of the two ears 
frequently also appears ; the writer has found this difference in about 
two-thirds of ears examined, no relation appearing between length of 
ears and symmetry of face. 

No reason appears for deeming this asymmetry of the face, even 
when verified by measurement, a sign of degeneration. 1 

The variety of facial asymmetry shown in Fig. 4 is not easily ex- 
plained as a mere twist in the skull or as a preponderance of material 
on one side. It will also be noticed that the ears, though fairly well 
formed, are badly set (too low). Perhaps complete knowledge of the 
subject would place this case in quite another category from the others. 
Until such knowledge shall have been acquired, are we justified in 
classing all as degenerates ? It is true that Dante has been reckoned 
among the degenerates, 2 but it would be reasoning in a circle to deem 
on this account his cranial asymmetry, or, in fact, any of the peculi- 
arities on which the diagnosis may have been based, signs of degen- 
eracy. 

Unusual size of lips represents a less common, but equally insig- 
nificant, deviation from the average normal. This peculiarity has 
recurred in one royal family with sufficient regularity to be named the 
" Hapsburg lip." (Figs. 5 and 6.) 

Another facial peculiarity is undue prominence of the lower part 
of the face. If a horizontal line be drawn from the external auditory 
meatus to the junction of the inferior nasal spine with the alveolar 
process (Broca's horizontal), and is cut by a line from the root of the 
nose, the facial prominence is shown by the varying angle between these 

1 Dwight (Seribner's Monthly, April, 1891) states that the right eye is generally 
the higher. He shows that the left eye of the Venus de Milo, photographed behind 
a framework, appears higher, and questions if this is not characteristic of left- 
handedness. In a personal communication he has assured the writer that he 
regards asymmetry of the skull and face, not as degenerative, but due to mechanical 
causes. It is interesting to note in this connection that among the many hundred 
inmates of the Massachuseets School for Feeble Minded, observed with reference 
to this point by the Superintendent, Dr. Fernald, as well as by the writer, facial 
asymmetry was found no more marked or prevalent than among average individuals. 

2 Lombroso, The Man of Genius. 1901. 



i 




Fig. 1. — Common form of facial asymmetry, but unusual in degree. 




Fig. 2.— Bust of Filippo Strozzi by Baudetto da Maiano. (Berlin Museum.) Right side of face 

appears longer. 




Fig. 3.— Bust of Dante. (From bronze in Naples Museum.) Left face appears longer. 




Fig. 4. — Less common form of facial asymmetry. 




Fig. 5.-The "Hapsburg lip," as shown by Albert, Archduke of Austria. An example of deviation, 

not degenerate. 




Fig. 6.— The same family peculiarity as shown by Leopold I. 






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DEGENERACY 953 

lines, or by the angle between the latter line and a vertical dropped 
from the root of the nose to the horizontal. 

The accompanying old print (Fig. 7) shows the deviation of the 
facial angle of Ferdinand VII. from the ideal, as represented by the 
profile of Bentham. It seems probable that this is classed more appro- 
priately under the stigmata of degeneration than simple facial asym- 
metry, though even this point is not really established. It was stated 
in the original legend that this represented the prognathism generally 
found in kings ! 

The illustration of Charles V. of Spain (Fig. 8) presents a further 
example of the Bourbon prognathism. 

In the case of Charles V. there was also projection of the lower 
teeth, and it is said this malformation of the jaw seriously impeded 
mastication. 

Ears badly set, asymmetrical, of unusual shape, lacking normal 
markings and normal parts, fall into the category of deviations which 
have been grouped as degenerative, but like most of the so-called stig- 
mata, each peculiarity deserves separate study before accepting this 
sweeping dictum. For example, ears set too low, or lacking lobes and 
markings, may, perhaps, be degenerative, while a prominent antihelix 
or a moderate difference in length may have no such significance. 

It is not, however, the purpose of this paper, to group this long list 
of deviations with regard to their bearing, or lack of bearing, on the 
question of degeneration. The illustrations cited will suffice to show 
that the estimation of their varying significance requires detailed and 
exhaustive study. This study is of practical importance in that it 
imparts knowledge regarding the constitution of the individual, a 
knowledge which helps the physician to weigh symptoms. Whichever 
of these deviations, alone or in combination, represent degeneration, 
it is true that they are generally more marked and more abundant in 
the insane, the criminal, and the idiotic, than in the well-balanced and 
the moral. This is, in fact, the important argument for attributing to 
all deviations the stigma which belongs only to a part, and to that part 
in an uncertain degree. It is also true that signs of deviation are com- 
mon among those possessing signs of inherited nervous and mental 
instability as well as hysteria, epilepsy, hypochondria, and other psychi- 
cal and physiological evidences of constitutional peculiarity or defect. 
In fact, these tendencies have been included in the list as psychical 
and physiological stigmata of degeneration. 

Given, therefore, a case in which any of these symptoms are sus- 
pected of resulting from eye-strain, it behooves the practitioner to be 



954 THE EYE AND NERVOUS SYSTEM 

on the lookout for other signs, both physical and mental, of deviation, 
for the more he finds, the greater is the probability that the symptom 
in question is due, in part or in whole, to the constitutional makeup of 
the individual, rather than simply to reflex irritation. 

THE BEARING OF CONSTITUTIONAL DEVIATION UPON THE 
ESTIMATION OF EYE-STRAIN SYMPTOMS. 

The study of the relation between eye-strain and symptoms refer- 
able to the nervous system has been handicapped by the apathy, not to 
say incredulity, on the part of the patient, general practitioner, neurolo- 
gist, and sometimes even oculist, regarding the variety and extent of 
symptoms which may be produced by the continuous, though apparently 
insignificant, irritation resulting from moderate refractive error. (See 
Chapter XVIII.) 

On the other hand, before predicting relief of nervous symptoms 
by correction of refraction, one definite factor in diagnosis must be 
carefully estimated, namely, the constitution of the individual. In a 
considerable number of cases the error of refraction is merely one evi- 
dence of a faulty organism, among other signs of which appear head- 
ache, the invalid habit, vertigo, indigestion, irritability, and allied 
nervous states, to say nothing of choreic movements, epilepsy, and other 
motor phenomena whose relation to eye-strain is not yet definitely deter- 
mined. While many of these symptoms are sometimes solely dependent 
upon eye-strain, hence susceptible of amelioration by glasses, and while 
the adjustment of glasses is often followed by gratifying results even 
in the constitutionally defective, a certain proportion of cases will baffle 
the most skilful and painstaking ocular treatment. 

With a view of emphasizing the practical bearing of this subject, 
it seems appropriate at this point to refer to a few of the most impor- 
tant and constant symptoms requiring analysis by the neurologist and 
the oculist, with reference to eye-strain as an etiological factor. 

The most constant and important of these conditions is cephalalgia, 
a symptom frequently relieved, and even cured, by simple correction of 
refractive error. To Dr. S. Weir Mitchell is due the credit of first call- 
ing general attention to this relation, a relation apparently already rec- 
ognized in practice by various oculists. 

It is, however, not unusual that a sufferer from persistent headache 
is forced to apply, in turn, to the oculist, the rhinologist, and the gyne- 
cologist. Examination discloses, successively, an error of refraction, a 
deviated septum, or projecting turbinate, and a misplaced or otherwise 
abnormal uterus ; but each successive special treatment, though encour- 



DEGEXEKACY 955 

aged by definite pathological findings, results in only temporary and 
insignificant relief of the headache. In such a case the underlying 
constitutional defect has been overlooked. This headache is often 
vaguely localized, often vertical, is less apt to be limited to the fore- 
head and the occiput, and is less often distinctly migrainous (see 
Chapter XVII.) in seat and character than that of the purely reflex 
ocular variety. 

The exact nature of the constitutional headache cannot be deter- 
mined. It seems improbable that there is a definite physical basis. In 
such event we are forced to resort to the unsatisfactory expression 
" functional." In some cases the description of suffering is so much 
at variance with the appearance and demeanor of the patient that the 
examiner is forced to the conclusion that the so-called suffering is an 
interpretation put by the patient upon what, in another, if noticed at 
all, would be merely a discomfort ; in other words, that the normal 
pressure of part upon part of the cranial contents, or the normal changes 
in circulation of the various fluids, or the sensation accompanying nor- 
mal mental effort, phenomena unnoted by the average individual, may 
be acutely and uncomfortably felt by the highly sensitive possessor of 
the psychopathic temperament. 

A well-recognized analogy is found in tinnitus aurium, a symptom 
generally due to aural disease, and often susceptible of relief by aural 
treatment, but resulting sometimes (in the morbidly sensitive or hys- 
terical individual), from the mere irritability of the sensorium, unable 
to accustom itself to the physiological impressions received by the 
auditory nerve. 

This variety of headache has been described and classified by writers 
on degeneracy as constitutional headache, and must be recognized as 
a sign of defective nervous makeup, of which the refractive error and 
the nasal and pelvic anomalies furnish merely contributory evidence. 

Another symptom not infrequently relieved by glasses is nervous 
irritability. Here again the practitioner should strive to distinguish 
between simple nervousness produced by continuous peripheral irrita- 
tion, and the various mental peculiarities resulting from inherent, and 
inherited, tendencies. The practitioner is often made aware of the 
mental attitude of patients of the latter class by a peculiar fussiness, 
by complaints of the temperature of the room, by a call for a fan, a 
glass of water, the shading of a light, the closing of a window. They 
often adopt an antagonistic attitude, and question the need of the 
various steps in the examination. Such patients, while complaining 
of most excruciating torture, are often unwilling to make the sacrifice 



956 THE EYE AND NEKVOUS SYSTEM 

of wearing glasses for its relief, or if they make the trial are unable 
to accustom themselves to the bows over the ears, the sight of the rims, 
or the reflection from the glasses. The efforts of the oculist are still 
further handicapped by the inability of such patients to relax the mus- 
cles of accommodation after the adjustment of glasses. They are also 
peculiarly prone to attempt extension of vision beyond its legitimate 
limits, and to look from side to side, though the latter tendency may 
be, in a measure, somewhat counteracted by terescopic or toric lenses. 

While the psychopath describes his headache in most extravagant 
terms, no corresponding signs of physical suffering appear. Though 
complaining of utter collapse, such a patient will often complete a 
long examination with smiling countenance and showing less sign of 
exhaustion than the examiner. The history in such cases will often 
disclose also hypochondriacal tendencies, morbid fears, antipathies, 
hyperconscientiousness, or mysophobia (fear of contamination). A 
combination of mental traits pointing in the same direction has been 
well summarized by Soukhanoff 3 under the " Ideo-Obsessive Consti- 
tution." 

Individuals with this peculiarity are easily recognized. They are 
" over-scrupulous, disquieted over trifles, indecisive in action, and anx- 
ious about their affairs. They are given early to morbid introspection, 
and are easily worried about their own indispositions or the illnesses 
of their friends. They are impressionable, possess considerable amour 
propre, and are easily offended; but as they are usually reticent they 
hide their resentment and are often secretly ashamed of those bizarreries 
peculiar to their disposition. They are often timorous and apprehen- 
sive, and prone to pedantism. The moral sentiments are pronounced 
in most cases, and if they are, as a rule, somewhat exigent and egotistic, 
they have a lively sense of their own defects." 

Hypochondria has been made a special subject in another chapter 
(XVI.), but deserves further brief mention in this connection. Hypo- 
chondria implies undue attention to symptoms, and undue alarm con- 
cerning them ; it may exist alone or in combination with other diseases, 
organic or functional. This tendency shows itself, as a rule, early in 
the investigation. The visit of the hypochondriac is apt to be preceded 
by a complete history of his case in writing. He is likely to consult 
memoranda in the elaboration of this history, and to describe his symp- 
toms with great particularity. He is prone to describe his sufferings 

3 Rev. Neurolog., Juin 30, 1903, p. 613. Abstract in Review of Neurology and 
Psychiatry, October, 1903, p. 703. 



DEGENERACY 957 

in anatomical terms ; for example, instead of stating he has a pain in 
the side, he locates the pain in the lung, the heart, or the intercostal 
nerve. He comes to the examiner with a preconceived and unshakable 
diagnosis of his own case, and in its defence exhibits great argumenta- 
tive ingenuity. He is generally anxious and willing to try a new 
remedy, usually with the result of temporary improvement. After the 
patient's departure the physician may expect to receive letters contain- 
ing further elaboration of symptoms. 

These psychoneuroses, still often loosely included under the title 
of neurasthenia, 4 are not to be mistaken for results of eye-strain, though 
marked refractive error is extremely likely to appear; and while no 
effort should be spared to contribute to the comfort of the patient by 
removing every possible source of reflex irritation, the chance is remote 
of overcoming inherent tendencies. 

Another condition for the relief of which glasses are not infre- 
quently recommended is epilepsy. The repeated failure of this pro- 
cedure is undoubtedly due to the fact that in the vast majority of cases 
this disease represents a constitutional defect in the cerebral mechanism. 
(Compare Chapter XVIII.) 

If experience had shown that overuse of other muscles than those 
of the eye produced generalized convulsions with impairment of con- 
sciousness, the burden of proof would be upon those denying that 
epilepsy is a common result of eye-strain, but in the absence of such 
evidence overwhelming proof is required to fortify the position of those 
who support this proposition. 

In the overuse of other muscles (for example, those of the forearm 
and hand concerned in writing), if spasm appears it is practically 
limited to the muscles involved; there is certainly no generalized 
spasm; nor is there a suggestion of impaired consciousness, the one 
essential symptom of epilepsy. In eye-strain the analogue of the local 
cramp is found in the spasm of accommodation, a well-recognized 
symptom. It is true that the analogy between eye-strain and writers' 
cramp is not complete in every particular, inasmuch as the pain of 
writers' cramp, in contradistinction to that of eye-strain, is usually 
limited to the overused member, and generally appears during, or im- 
mediately after, the overuse of the part. The divergence from this 
rule in the case of pain resulting from eye-strain is, however, insig- 
nificant compared with the introduction of such new elements as gen- 
eralized convulsions and impairment of consciousness. 



4 See Dana, The Partial Passing of Neurasthenia. 



958 THE EYE AND NERVOUS SYSTEM 

In one case of epilepsy under the care of the writer an interval 
of sixteen months followed the correction of a very obvions refractive 
error. At the end of this period, however, the attacks returned in spite 
of the continued efforts of a particularly skilful refractionist. In prac- 
tically all the epileptics coming under the writer's observation in the 
Neurological Department of the Massachusetts General Hospital and 
elsewhere, the question of refraction has been carefully considered, but 
its correction has yielded negative results beyond occasional variations 
in the duration of the intervals, variations too inconsiderable and too 
inconstant to offer proof of material benefit, and often to be regarded 
as a mere coincidence. 

Such experiences have led to scepticism regarding the cure of epi- 
lepsy by adjustment of glasses, and while search through the literature 
reveals a sufficient number of apparently ameliorated cases to preclude, 
perhaps, the final closing of this branch of investigation, it may at least 
be stated that the dependence, in any considerable proportion, of epi- 
lepsy on eye-strain, and its cure by refractive correction, is yet to be 
established. 

The prevalence of convulsive attacks in the epileptic patient after 
overloading the stomach has not infrequently led to the conclusion 
that the attacks were of reflex nature with digestive disorder as under- 
lying cause, but, while regulation of the diet often lessens the attacks, it 
signally fails, in the majority of cases, to reach the real seat of the 
trouble. Epilepsy is, then, like the refractive error and the indigestion, 
generally only another sign of constitutional defect. In point of fact, 
careful search through the family history of the epileptic will generally 
reveal, if not epilepsy, hysteria, or insanity, at least a prevalence of 
some of the morbid mental tendencies to which reference has been 
made in the consideration of constitutional headache and nervous irri- 
tability. 

In the attempt to relieve chorea, habit chorea, hysteria, and insanity, 
to say nothing of indigestion, vomiting, and insomnia by refractive 
correction, the effort must be made in each case, before making a prog- 
nosis, to determine how great a part is played in the production of the 
clinical picture, not only by other causes, but also by the inherent 
makeup of the individual. 

If there are signs by which constitutional instability can be detected 
at the outset of the examination, the knowledge of such signs will be 
of material aid in diagnosis and prognosis. Reference has already 
been made to the mental attitude sometimes characterizing these pa- 
tients. Compulsive acts will sometimes be noted ; frequent grunting, 




Fig. 8.— A further illustration of the prognathism of the Bourbons, as shown by Charles V. of Spain. 



r% 




Fig. 9.— Ears asymmetrical, badly shaped, badly set. (From Massachusetts School for Feeble Minded, 

through kindness of Dr. Fernald. ) 




Fig. 10.— Absent lobe, crumpled helix, antihelix prominent and of unusual shape. Incisoura intertragica 
broadened, concha large. (Same source as Fig. 9. ) 




Fig. 11. — Helix narrow, thin, and irregularly shaped, with suggestion of Darwinian tubercle; antihelix 
prominent ; abnormal fold in lobe. ( Same source as Fig. 9. ) 



DEGEKEKACY 959 

blinking, or sniffing, mannerisms in speech and deportment are sugges- 
tive. Among the physical peculiarities may be noted deviations in form 
of the skull and face, 5 unusual shape, size, or position, of the orbits, 
narrow palpebral fissure, excessive prominence of the forehead, prog- 
nathism and projection of the lower teeth (Figs. 7 and 8), peculiarity 
in shape of the palate, peg-shaped incisors, hair upon the face (in the 
female), ears badly shaped, unlike, or badly set (Figs. 9, 10, and 11), 
and curvature of the spine. It must not be understood that the physical 
and mental stigmata always go hand in hand. 

DEVIATION. 

The various constitutional defects and peculiarities have been 
summed up, as has been said, rather loosely, under the term degeneracy 
or degeneration. These stigmata include every conceivable variation 
from the average normal (Dana, Church and Peterson, and others), 
ranging all the way from such innocuous peculiarity as a difference in 
color of the two eyes, to perversion and imbecility. It has not, how- 
ever, been yet established that every sign of deviation from the average 
normal is degenerative, and it seems to the writer 6 desirable to name 
the phenomena signs of deviation and to call their possessor deviate, 
or a deviate, as the case may be, limiting the term degeneration only 
to such deviations as obviously imply deterioration, either of the indi- 
vidual or of the race. The nomenclature which has hitherto obtained 
in anthropological study, largely through the writings of !N*ordau 7 and 
of Lombroso, 8 if applied to the vegetable kingdom, would without 
investigation stigmatize the four-leaved clover as degenerate. 

The illogical nature of the present method is illustrated by the 
statement of Lombroso 9 that four per cent, of normal individuals have 
five or more signs of degeneration. Would it not be equally descriptive 
and more accurate to say that this percentage of normal individuals 
have signs of deviation ? 

BIOGRAPHICAL STUDY OF CONSTITUTIONAL 
DEVIATIONS. 

A careful study of the lives of various men prominent in literary 
and scientific work would indicate that they were hampered by eye- 

5 The usual form of asymmetry, previously discussed, has probably no bearing. 

6 Walton, The Prevailing Conception of Degeneracy and Degenerate, with a 
Plea for Introducing the Supplementary Terms Deviation and Deviate. Boston 
Medical and Surgical Journal, January 21, 1904. 

7 Degeneration, Max Simon Nordau. New York: Appleton & Co., 1895. 

8 L'uomo delinquente ecc, 1889. 

9 Lombroso, in Twentieth Century Practice of Medicine. New York. 1897, p. 381. 



960 THE EYE AND NERVOUS SYSTEM 

strain. (Gould. 10 ) But the same study made with due consideration 
for possible etiological factors other than eye-strain, for the mental and 
physical discomforts under which they labored, shows that the problem 
is not so simple. It is a question whether enthusiasts in other branches 
of medicine might not find ample evidence of the injurious effects of 
pathological states involving other organs. Might not, for example, 
the dentist attribute De Quincey's indigestion to his defective teeth ? 
Or, again, might not the diathetic enthusiast find sufficient evidence 
in Carlyle's case to claim an excess of uric acid as the cause of his 
unfortunate disposition? However this may be, the history of such 
cases is apt to reveal signs, physical, mental, or both, of constitutional 
peculiarity. Carlyle in his boyhood was shy, proud, pugnacious, with 
strong affections and with equally violent antipathies. In later life he 
declared war upon the neighbor's poultry, and had constructed a room 
for his labors, proof against sound. De Quincey was a man of diminu- 
tive stature, given to day-dreaming before he became addicted to his 
unfortunate habit, the inability to resist which may quite as plausibly 
be attributed to constitutional defect as to the discomfort caused by 
eye-strain. 

The exalted character of the many celebrities cited by Lombroso, 11 
in establishing his conclusion that genius is a degenerative psychosis, 
accentuates the necessity of supplementing the word degeneracy by 
some less opprobrious, while equally descriptive and inclusive term. 
Even the fact that many of these individuals showed decided deteriora- 
tion in the physical or moral scale does not militate against adopting 
a term for the classification of their variations from the normal before 
drawing conclusions regarding the significance of those variations. In 
dealing with genius it is well to remember also that the impression pre- 
vailing in popular, not to say scientific, circles, that moral qualities 
stand in an inverse ratio to intellectual endowment, has been contro- 
verted by the statistical investigations of Woods. 12 

Etiology. — The varied etiology of the so-called stigmata of degener- 
ation would already indicate great diversity in their downward, nega- 
tive, and even occasionally upward values. The wide and indiscrim- 
inate use of the word degeneration has led to conflicting views of the 
origin of the stigmata. Critical review of this etiology is as far beyond 
the ability of the writer as beyond the scope of this treatise, but brief 

10 Biographic Clinics. Philadelphia : P. Blakiston's Sons & Co., 1903. 

11 The Man of Genius. London: Walter Scott, 1901. 

12 F. A. Woods, The Correlation between Mental and Moral Qualities. Popular 
Science Monthly, October, 1903. 



DEGENERACY 961 

mention of some of the suggested causes will be in place. From Nor- 
dau's point of view it is natural that disease, privation and excesses in 
the ancestry should be regarded the main factors in the production of 
degeneracy, and it is doubtless true that such causes result in enfeeble- 
ment, undersize, and defective resistance in the progeny ; but according 
to modern scientific views the presence or absence of inherent charac- 
teristics transmitted by the germ are independent of such influences. 
In other words, acquired characteristics are not transmitted. 

The present anomalous use of the term degeneration includes among 
the deviations from the average normal, even such evolutional spurts 
as unusual development of Broca's convolution in the individual pos- 
sessed of unusual linguistic aptitude (Lombroso). This use of the term 
renders inadequate the etiology of Nordau. 13 Paul Jacoby 14 has 
endeavored to show that selective tendency in aristocratic circles is 
answerable for sterility and family decadence. Similarly, Ireland 15 
has laid stress on indolence and the " insanity of power" as causing 
degeneracy in royal families. The studies of Woods 16 .clash with these 
conclusions, for he has shown by the analysis of many royal families, 
in which the surroundings and manner of life remain practically con- 
stant factors, that while some families have deteriorated others have 
advanced, the variations in the individuals and families depending pre- 
eminently on the combinations of characteristics inherited. 

Classification of Causes of Deviation. — I. Modern embryological in- 
vestigations have shown that the inherent and therefore hereditary char- 
acteristics transmitted through the germs entirely dominate the struc- 
ture of the progeny. 11 Just what characteristics the child will inherit 
from each parent cannot be foretold ; still less can it be predicated what 
combinations of more remote ancestral traits will appear in any given 
progeny. For the study of this fundamental branch of the subject the 



13 Prior to Nordau's more popular treatise. Morel (Traite, etc., 1857, p. 17) 
had dwelt upon the constitutional basis for insanity. 

14 Etudes sur la Selection dans ses Rapports avec FHeredite chez l'Homme, 
Paris, 1884. 

15 The Blot upon the Brain. G. P. Putnam's Sons, New York. 1896. 

16 Mental and Moral Heredity in Royalty. Popular Science Monthly, August, 
1902, to April, 1903. 

17 Wilson summarizes the present view of maturation of the sexual elements 
thus: "In both sexes, the final reduction in the number of chromosomes is effected 
in the course of the last two cell divisions by which the definite germ cells arise, 
each of the germ cells thus formed having but half the usual number of chromo- 
somes. In the female but one of the germ cells forms the ovum proper, while 
the other three, known as the polar bodies, are minute, rudimentary and incapable 
of development." " In the male, on the other hand, all four of the cells become 

Gl 



962 THE EYE AND NEKVOUS SYSTEM 

reader is referred to Weissmann, 18 Minor, 19 Wilson, 20 and other author- 
ities, and for experimental studies in transmission, to the researches in 
heredity inspired by the discovery of Mendel's law (de Vries, Correns, 
Tschermak, Bateson, Castle.) 

Weissmann's view, that acquired characteristics cannot be transmit- 
ted, is accepted by the majority of biologists 21 and tends still further to 
accentuate the importance of the original nuclear substance in the trans- 
mission of stigmata of deviation, though constitutional enf eeblement of 
varied forms in the progeny may well result from deleterious influences, 
such as defective nutrition of the germ in utero, due to the progenitors. 

Inherited tendencies certainly constitute an important factor in 
the production of the psycho-neuroses, hysteria and insanity. 

(a) The tendency of family characteristics to recur after a period 
of latency is known as atavism, sl phenomenon which would seem espe- 
cially prone to occur in the offspring of extremely dissimilar parents. 
Darwin found, for example, that by crossing a black Spanish and white- 
silk fowl offspring could be produced with red plumage resembling 
that of the wild ancestor common to both races. (Mercier. 22 ) Ei- 
baud 23 denies degenerative tendency in this class of cases, and suggests 
that in man Polydactyly (supernumerary fingers) and polymastia 
(many breasts) come under the same category, the former serving as a 
reminder of the numerous swimming rays of the fishes, the latter of 
the multiple breasts of mammals inferior to man. Whether Bibaud's 

functional spermatozoa," — " the polar bodies are therefore to be regarded as abor- 
tive eggs." Again "if it" (the process of reduction of chromosomes) "did not 
occur, the number" (of chromosomes) "would be doubled in each succeeding 
generation through union of the germ cells." He quotes Weissmann's explanation 
of the reason for the reduction as preventing the excessive accumulation of differ- 
ent kinds of hereditary tendencies or germ plasms. 

18 Ueber Vererbung, Jena, 1883, 1885. Die Continuitat des Keimplasmas u. s. v. 
Jena, 1883, 1885. Ueber die Zahl. der Richtungs Korper. u. s. v. Jena, 1887. Essays 
on Heredity, Oxford, 1891, 1892. 

19 Human Embryology. Wm. Wood & Co., N. Y., 1892. 

20 The Cell in Development and Inheritance. The MacMillan Company, 1896. 

21 Minot states authoritatively that the "hypothesis of pangenesis" (Darwin's 
theory that the egg consists of a multitude of germs or gemmules thrown off from 
each part or unit of the parent body, each gemmule possessing the properties which 
the unit had when they were thrown off) " both in its original form and in all its 
subsequent modifications, has been definitely set aside" ; again, " the child is like 
the parents because its organism is regulated by not merely similar, but by some 
of the same chromatin as that of the parents," adding that possibly the more 
general term nuclear substance would be, perhaps, preferable in place of chromatin. 

(Loc. cit. p. 90.) 

22 Mercier, Sanity and Insanity. London : Walter Scott. 

23 Abstracted from Revue Scientifique in the Literary Digest, December 5, 1903. 



DEGENERACY 9G3 

views receive general acceptance or not, this line of thought will serve 
to remind us that the absence of neurotic history in the immediate 
family does not preclude such inheritance from a distant ancestor. It 
is possible that Mendel's laws of dominance and recession (latency) 
may prove of aid in determining the reappearance of deviative signs. 

(&) The view long accepted without question that the effect of 
consanguineous marriage in itself is harmful is voiced by Mercier, who 
states that while a certain degree of dissimilarity between parents pro- 
duces the best offspring, too great similarity in parents tends to enfee- 
blement and idiocy, while too great dissimilarity, if productive at all, 
tends towards wildness in the offspring. It is, he says, on account of 
the deleterious effect of parental similarity that marriage between near 
relatives is undesirable, but if it should chance that cousins obtain their 
prepotent qualities from different ancestors rather than from the same 
ancestor, the liability to deterioration in the offspring is lessened. 

If the prepotent qualities are represented by heavy lines in Eig. 12, 
the cousins C, C, children of brothers B, B, inherited, in (a), pre- 

G G' G f 

C G G C 



(«) 



(&) 
Fig. 12.— After Mercier. 



potently the characteristics of this common (paternal) grandfather, G, 
hence their marriage, according to Mercier, would be unwise. One of 
the cousins in (5), however, derives prepotent qualities from the mater- 
nal grandparent (G 1 ), so that the risk to posterity is reduced to the 
minimum. 

This view of the deleterious effects of close intermarriage is not 
borne out by the extended investigations of A. H. Huth, 25 excepting 
in so far as the cousins inherit in common vicious tendencies. 

Whatever evil effects may follow continued inbreeding, they may 
be materially reduced by the introduction of new blood after several 
generations, as in the case of the most notable instance on record of 
continued consanguineous parentage, namely, that of the Ptolemies. 
(Mercier.) The repeated recurrence of the Hapsburg lip (Figs. 5 



24 Castle, Proceedings Amer. Acad, of Arts and Sciences, vol. xxxviii. No. IS. 
With bibliography. Cambridge, Mass.. January, 1903. 
- 5 The Marriage of Near Kin, etc. London, 1SS7. 



964 THE EYE AND NERVOUS SYSTEM 

and 6) furnishes another illustration of the effect of (c) pure selective 
tendency in the parents, an illustration which it would not be just to 
stigmatize without further analysis as degenerative. (Woods.) It is 
also of interest in this connection to study the collection of Meige, 26 
illustrating the degenerate types in art. This collection includes cases 
of myxcedema, achondroplasia, and rhachitic diathesis, as well as 
individuals showing marks of moderate deviation from the average 
normal among the subjects treated by various prominent painters, nota- 
bly Velasquez. 

In the production of insanity, heredity and stress are essential and 
variable forces ; the greater the instability of the nervous system the 
less the stress required. (Mercier). Similarly, the character of an 
individual depends on both heredity and environment. Environment 
and education can do much to modify inherited characteristics; but 
education and surroundings can no more entirely remove the evidences 
of psychopathic endowment than they can efface the anatomical signs 
of deviation. 

II. In the causation of such degenerative conditions as hydro- 
cephalus (Eig. 13), Vaschide and Vurpas 2T have called attention to 
the infective processes operating upon the developing embryo. Upon 
this etiological factor the writer is not qualified to comment, further 
than to suggest that in comparison with the long list of stigmata cited, 
it seems of comparatively limited application. 

III. Still another cause of deviation may be found in mechanical 
influences affecting tine embryo. Dr. Whitney (Curator of the Warren 
Museum) has suggested amniotic adhesions and amputation by the 
foetal cord (Figs. 14 and 15). 

Possibly the common asymmetries of the face should fall under this 
head. 

IV. Certain anomalies, such as congenital absence or peculiar 
growth, of certain parts, whether due to absence of certain elements 
in the germ, or to their later disappearance or faulty development, have 
been termed freaks of nature. The fact that they are not yet explained 
does not preclude a definite etiology. The mandible hand (Fig. 16) 
must be placed in this category. 

This class of deviations sometimes recurs in families. This was 
the case in the defective lobe in Fig. IT. 



26 La Presse Medicale, Juillet 15, 1903. 

27 Ann. di Nevro, February 1, 1903. Abstr. in Review of Neurology and Psy- 
chiatry, June 1, 1903. 





Fig. 13.— A remarkable degree of hydrocephalus. Fig. 14.— Amputation by strangulation of 

(Copyright, 1S88, by Langill, Hanover, N. H. ) total cord. ( From cast in Warren Museum. ) 




V 













Fig. 15.— Results of amniotic adhesions. (From plates in Ahlfeld, Misbildung des Meusohen 1880. ) 




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Fig. 19.— Group of microcephalic inmates of Massachusetts School for Feeble Minded. (By kindnes 

of Dr. Fernald.) 




Fig. 20.— Group of macrocephalic inmates of the same institution. 




Fig. 21.— Casts of palates from the collection of Dr. Charming. They illustrate varieties in shape, 
width, height, and symmetry; one shows "torus palatimus ;" none are absolutely normal. All are 
taken from the feeUle minded. 




1 




Fig. 22.— Inmate of the same institution, aged 23. Absence of hair on face and pubes, hypospadias, 
infantile penis, cryptorchismus, muscular insufficiency. 



DEGENERACY 965 

V. Tlie investigations of Thompson tend to show that mechanical 
Influences affecting the development of the infant should be given a 
place. He maintains that the shape of the sknll depends largely on 
the cranial capacity, and in some degree also upon the strength of the 
temporal muscle. This investigator replaced the calvarium by a rubber 
bag to represent the membranous condition of this part of the skull 
during its developmental period ; moderate inflation of the bag pro- 
duced a shape resembling that of the ape's skull with slight frontal 
development, and, viewed from in front, a shape suggested by that of 
the idiotic child shown in Eig. 18 (for which I am indebted to Dr. 
Waterman). 

Great distention produced the normal adult shape. This differ- 
ence was accentuated by weight-bearing cords attached to the bag in 
the region of the temporal muscle. The question is complicated by 
the difference in leverage under which the temporal muscle works with 
different shaped jaws, the long arm of the lever being the condylo- 
symphyseal measurement, the short, the condylocoronoid measurement. 
The similarity in shape of microcephalic and macrocephalic skulls re- 
spectively in the groups of feeble minded (Figs. 19 and 20) seems to 
lend weight to Thompson's hypothesis. 

VI. It has already been stated that constitutional disease, depriva- 
tion, and excesses in the ancestry may so far impair the nutrition of 
the germ as to cause undersize, enfeeblement, and lessened resistance 
in the offspring though not influencing its inherent characteristics. 

VII. Certain glands have a distinct, though not thoroughly under- 
stood, influence on the nutrition of the body; hypertrophy or defect 
of these glands produce marked variation in its growth. Giantism 
is found associated with enlarged pitituary gland, and results perhaps 
from its oversecretion ; possibly dwarfism implies diminution of this 
secretion, though this point has not been investigated as far as the 
writer is aware. Acromegaly, a condition in which the face, jaws, 
hands, and feet become enormous in later life, after the bones have 
acquired their length, is also found associated with disease of this 
gland and deserves a place in the same category with giantism. De- 
fective glandular tissue in the thyroid produces cretinism in infancy, 
myxoeclema in adult life. 

VIII. Dr. Minot (to whom I am indebted for critical revision of 
the portions of this chapter which bear on embryology) suggests thai a 
fundamental etiological factor should be added, namely, such lack of 
development as is evidenced by harelip. Tie attributes I he erroneous 
use of the word degeneration mainly to failure to recognize the essential 



966 THE EYE AND NERVOUS SYSTEM 

steps in degenerative processes, embraced under cytomor •■pilosis. This 
word lie uses " to designate comprehensively all the structural modifi- 
cations which cells, or successive generations of cells, may undergo, 
from the earliest undifferentiated stage to their final destruction." 28 
In other words, existence of protoplasm and its growth are prerequisites 
for degeneration. A deposit of horny material in the skin may then 
be correctly classed as degenerative, in contrast to such conditions as 
harelip. Nor can degeneration of the race be established upon signs 
which do not indicate degeneration in the individual. 

It would seem, then, that we must include among the possible causes 
of the so-called stigmata of degeneration (which include signs of devia- 
tion only), at least 




Fig. 23.— Unusual development of the breasts in a negro boy. (Case of Dr. Spill er.) 

I. The potential variations from the average normal contained in 
the parent germ, including the results (a) of atavism, (b) of parental 
similarity, and (c) of selective tendency on the part of the ancestry. 

II. Intra-uterine infection. 

III. Mechanical injury during intra-uterine life. 

IV. The absence or peculiarity in the germ of certain elements, 
or their disappearance or anomalous development, without traceable 
inherited tendency or other known explanation. To this class of stig- 
mata the unsatisfactory and doubtless inadequate term " freak of 
nature" (lusus naturae) has been applied. 



28 A Laboratory Text-Book of Embryology. Philadelphia : P. Blakiston's Son 
& Co.. 1903. 



DEGENERACY 967 

V. Mechanical influence exerted during infancy. 

VI. Deleterious influences and habits in the ancestry (productive 
of enfeeblement, undersize, and lessened resistance in the progeny but 
not altering the essential potential characteristics transmitted by the 
parent germ). 

VII. Absence or hypertrophy of certain glands (pituitary, thyroid) 
which have a nutritional influence. 

VIII. Arrest of development such as is seen in harelip and similar 
defects. 

List of Stigmata. — The following list comprises a large number, 
though by no means all, of the pathological conditions and anomalies 
which have been cited by various authorities as stigmata of degenera- 
tion. It is obviously more appropriate to classify a considerable num- 
ber of them as signs merely of deviation. Some of those whose pres- 
ence, either alone or in combination with others, have little if any 
bearing on the question of true degeneracy are starred. 

ANATOMICAL STIGMATA. 

Cranial Anomalies. 

Asymmetry of skull, especially frontal bone. 

Macrocephalus. 

Microcephalus. 

Platycephalus or chemocephalus (flat). 

Leptocephalus (narrow). 

Oxycephalus or acrocephalus (steeple shaped). 

Plagiocephalus ( oblique ) . 

Scaphocephaly (keel shaped) . 

Trigonocephalus or trigonal (pointed anteriorly). 

Short parietal arc. 

Short frontal are. 

High prominent forehead in women. 

Heavy jaws. 

Great or unequal prominence of malar bones. 

Lemurian hypophysis. 

Prognathism. ~\ Unusual projection, straightness or re- 

Orthognathism. I cesgion of the lower face 

Opisthognathism or retrognathism. \ 
Large frontal sinuses, small orbit. 

Crania progenaea (lower teeth projecting beyond upper, and inferior maxillary 
angle obtuse). 
*Facial asymmetry. 
Anomalies of the eye. 

Narrow palpebral fissure. 
Micropthalmus. 
*Flecks on the iris. 
* Chromatic asymmetry of the iris. 
Albinism. 
Congenital cataracts. 



968 THE EYE AND KEKVOUS SYSTEM 

Cranial Anomalies ( Continued ) . 
Anomalies of the eye. 

Pigmentary retinitis. 

Muscular insufficiency, strabismus. 
*Hypermetropia. 

Myopia. 

Astigmatism. 
Deformities of the palate. 

High and narrow (sometimes associated with adenoid hypertrophy). 

Horseshoe. 

Torus palantinus (longitudinal ridge along palatine suture). 

Gothic arch. 

Dome-shaped. 

Flat-roofed. 

Hip-roofed. 

Asymmetrical. 
Dental xlnomalies. 

Badly set and badly nourished. 

Small or peg-shaped lateral incisors. 
^Double rows. 

* Adventitious teeth. 
*Double crown. 
*Macrodentism. 

Microdentism. 

Projecting teeth. 

Badly placed or misplaced teeth. 

Striated transversely. 

Hutchinson's teeth. 
Anomalies of the nose. 
^Deviation of nose. 

Defective development of cartilage and tissue of alae (Gudden). 

Absent. 

Atresia of nasal fossa. 

Defective osseous development (nasus aduncus). 
Anomalies of the tongue and lips. 
*Macroglossus. 

Microglossus. 
*Bifidity of point. 

* Hare-lip. 
*Cleft palate. 

Anomalies of the ear. 
*Excessively long. 
*Excessively prominent. 
*Set too close to the head. 

Set too far back. 

Set too low. 

Absence of helix, antihelix, or lobule. 

Obliteration of markings. 

Absence of fissura intertragica. 

Too conchoidal (antihelix, crura, etc., too little marked and helix like 

rim of funnel ) . 
*Excessively large (absolutely or relatively). 

Too small. 

Lack of uniformity in width. 



DEGEKEKACY 969 

Anomalies of the Eak (Continued). 

Asymmetry of the two ears, general anomaly of left (Blainville ear). 
* Prominence of antihelix. 
*Adherent lobules (found in a large percentage of normal individuals). 

[Definite types of misshapen ears are described and named, for example, the 
Stahl, Nos. 1, 2, and 3; the Wildermuth; the Morel; the Cagot. Descriptions and 
illustrations of these varieties may be found in the text-book of Church & 
Peterson, p. 628 et seq.~\ 

Anomalies of the Limbs. 

*Left arm and leg longer than right. 
*Excessive length of arms. 
*Long fingers. 
* Polydactyly. 

Symphysodactyly or achistodactylus (joining of fingers). 

Syndactyly (web fingers). 

Ectrodactyly ( fingers wanting ) . 

Amelus or ecromelus (limb wanting). 

Phocomelus (segment of limb wanting). • 
*Megalomelus. 
*Megalodactyly. 

Oligomelus (excessive gracility). 

Oligodactyly. 

Anomalies of the Trunk and General Structure. 
Hernias. 

Malformation of the breasts and thorax. 
Dwarfism. 
Giantism. 
Infantilism. 
Femininism in men. 
Masculinism in women. 
*Spina bifida. 
Feebleness of construction. 
Lordosis. 
Scoliosis. 
Kyphosis ( Fere ) . 
Malformed coccyx. 
Thoracic asymmetry. 
Mammary development in males. 
Mammary absence or reduplication in females (polymastia). 

Anomalies of the Genital Organs. 

Small or deformed genitals. 

Hermaphrodism. 

Cryptorchismus. 

Hypospadias. 

Epispadias. 

Defect, torsion or great volume of prepuce. 

Imperforate meatus. 

Labia too large or too small. 

Clitoris large. 



970 THE EYE AKD KEKVOUS SYSTEM 

Anomalies of the Genital Organs (Continued). 
Labia minora hypertrophied. 
Folds between labia majora and minora. 
Labia minora pigmented, particularly in brunettes. 
Imperforate vulva. 
Artresion of vagina. 
Double vagina. 
Uterus bicornis. 
Atrophic uterus. 

Muscular Anomalies. 

Dystrophies. 

Unequal inervation of facial muscles on the two sides. 

Depression above glabella due to over-action of corrugators. 

Anomalies of the Skin. 

Polysarcia. 

Hypertrichosis (superiluous hair). 
*Premature grayness. 

*Precocious and abnormal hairy development. 
*Rudimentary tail. 

Glabrous chin (no beard). 

Vitiligo. 

Absence of nails or foetal state of nails. 

Melanism of skin. 

Pigmented or vascular nevi. 

Molluscum. 

Ichthyosis. 

Pigmented spots. 

PHYSIOLOGICAL STIGMATA. 

Anomalies of Motor Function. 

*Left-handedness. 
Retardation of learning to walk and talk. 
Tremors. 
Epilepsy. 
Nystagmus ( congenital ) . 

Tics. 

*facial spasm, 
habit chorea, 
tic convulsif. 

Anomalies of Sensory Function. 

Deaf-mutism. 

Neuralgia. 

Migraine. 

Constitutional headaches. 

Hyperesthesia. 

Anaesthesia. 

Blindness. 

Daltonism (color blindness, achromatopsia). 

Hemeralopia (night blindness) . 



DEGENERACY 971 

Anomalies of Sensory Function (Continued). 
Nyctalopia (day blindness) . • 

Concentric limitation of the visual field. 

Anomalies of Speech. 

Mutism. 

Defective speech. 
* Stammering. 
*Stuttering. 

Anomalies of Genito-Urinary Function. 

Sexual irritability. 

Impotence. 

Sterility. 

Urinary incontinence. 

Amenorrhoea. 

Anomalies of Instinct or Appetite. 
Mercyism ( gluttony ) . 
Rumination. 
Uncontrollable appetites (food, liquor, drugs). 

Diminished Resistance against External Influences and Diseases. 

Retardation of Puberty. 

Deficient Vital Activity of Organic Functions. 

Weak heart. 

Low arterial tension. 

Coldness of extremities. 

Flushing of extremities. 

General chills and flushes. 

Weak digestion. 

Constipation. 

PSYCHIC AND PSYCHO-NEUROTIC STIGMATA. 

Dementia prsecox. 
Compulsive insanity. 
Manic depressive. 
Melancholia. 
Hysteria. 
Hypochondria. 
Phobias. 

Psychopathic endowment. 
Invalid habit. 
Idiocy. 

Feeble-mindedness. 
*Eccentricity. 
Moral deliquency. 
Sexual perversion. 
Precocity. 

Overdevelopment of certain aptitudes. 
Paranoia. 
Ideo-obsessive constitution. 



INDEX. 



¥¥ 



Abducens nerve, 

intracerebral anatomy of, 16 

paralysis of, from meningitis, 16 

palsy of, 

symptoms of, 235 
with migraine, 717 
Aberration, 35 

varieties of, 36 
Abiotrophy, 545 
Accessorius nerve, 

cerebral portion of, 

intracerebral anatomy of, 18 

spinal portion of, 

intracerebral anatomy of, 19 
Accommodation, 

anomalies of, 

hysterical, 641, 640, 644 
pathogenesis of, 645 

determination of power of, 56 

failure of, with age, 
symptoms of, 778 

impairment of, with paresis, 484 

loss of, with multiple neuritis, 605, 608 
with tabes, 504 

measurement of, 38 

mechanism of, 37 

power of at different ages, 39 

painful, with hysteria, 640 

palsy of, with brain tumor, 404 

range of, 38 

spasm of, 

epileptic, 957 
Achromatopsia, 

(see Color blindness), 88 
Acoustic nerve, 

intracerebral anatomy of, 17 
Acromegaly, 

cause of, 559 

diagnosis of, 561 

differential, 564 

ocular signs of, 561 

Packard's case of, 560 

pathology of, 562 

symptoms of, 559 to 562 

treatment of, 564 

with pituitary disease, 426 
Agrammatism, 144 

cause of (theoretical), 147 

mechanism of, 144 

training in treatment of, 145 
Agraphia and allied conditions, 

cause and mechanism of, 139 

with migraine, 725 
Akotaphasia. 146 
Alcoholism, 

ocular signs of, 525-528 
Aleucopia, 87 
Alexia, 138 

causes of, 138 

treatment of, 142 

with brain tumor, 390 
Amaurosis, 

(see Blindness) 
Amaurotic, family idiocy, 

cause of, 534 



Amaurotic, family idiocy, characteristics of, 532 

history of, 532 

ophthalmoscopic signs with, 532 

pathology of, 535 

symptoms and course of, 535, 533 

treatment of, 536 
Amblyopia, central or muscular, 108 

cortical, 102 

crossed, 102 

exanopsia, 177 

feigned, 

tests to detect, 692 

hysterical, 621 

explanation of, 113 

simulation of, to sympathetic disease, 669 

monocular, 108 

pretended, 692 

uraemic, 177 

with alcoholism, 525 

with lead intoxication, 528 

with liver disease, 742 

with migraine, 700, 703 

with multiple neuritis, 603 

with paresis, 479, 486 
Amblyoscope test, 204 
Ammetropia, see Refraction anomalies 
Amnesia, 

color, 88, 101 

lesions causing, 122 
Amorphopia, 87 

Amyotrophic lateral sclerosis, 580 
Anaemia, 

pernicious, 

ocular and spinal signs of, 582. 583 

visual disturbances with, 741 
Anaesthesia, 

corneal, 322 

conjunctival, 171 
Anaphoria, 782 
Anisocoria, 314 
Anisometropia, 49 
Anomia, 

color, 101 

visual, 148 
Anophthalmus, 552 
Anopia, 

cortical, 

differential diagnosis of, 85 
Antimetropia, 49 
Aphasia, 

color, SS 

causes of, 101 

crossed, 135 

graphic, 

epilepsy with. 139 

optic. 148 

lesions, causing, 149 

teaching in treatment of, 143 

visual, 115 

treatment of, 142 
varieties of, US 

with brain tumor. 390, 398, 399 

witli migraine. 708. 72."> 
Apraxia, 
| sensory, 120 

visual. 118 

973 



974 



INDEX 



Aqueous humor of eye, 30 

Astasia abasia with exophthalmic goitre, 830 

Astereognosis, 

causes of, 122 

visual disorders with, 114 

with exophthalmic goitre, 830 

with brain tumor, 396 
Asthenic paralysis, 457 
Asthenopia, 

accommodation, 811 

causes of, 781, 814, 779 

constitutional, 815 

definition of, 750 

effect of, on neurasthenia, 779 
on refraction, 781 

facial expression in, 814 

from strenuous life, 779 

general, 811 

hysterical, 659, 640 

muscular, 781, 813 

neurasthenic, 672 
in children, 673 

reflex, 815 

symptoms of, 816 
Astigmia, 

of refraction, 46 

measurement of, 49 

varieties of, 47 
Ataxia, cerebellar, 393 

hereditary, diagnosis of, 475 

resemblance of, to Friedreich's 

ataxia, 550 
with small cerebellum, 550 

Friedreich's, 474 

diagnosis of, 475 
ocular signs of, 474 

locomotor (see Tabes) 

with brain tumor, 393, 400, 403, 404, 421 , 
429, 431 

with multiple neuritis, 602 
Athetosis, 

atrophy of cerebrum with, 549 
Atrophy, 

primary neurotic, 852 
Attention reflex, 304 
Auditory nerve, 

palsy of, 

with ocular palsy, 240 
Aurai (visual), 

with epilepsy, 732 

with migraine, 700 



B 

Bar reading test, 203 
Basedow s disease 

(see Exophthalmic goitre), 828 
Baudry's test, 693 
Blepharospasm, 336 

diagnosis, prognosis and treatment, 337 

epileptic, 733 

hysterical, 651 

tabetic, 502 
Blindness, alcoholic, 526 

anaemic, 741 

color (see Color blindness, 88) 

diagnosis,175 

epileptic, 734 

green, 88 

hysterical, 614 
bilateral, 614 
intermittent, 616 
nature of, 619 
unilateral, 616 

letter, 137 



Blindness, mind, 118 

causes, 122, 127 
numeral, 142 
object, 118 
paroxysmal, 466 
peripheral, 173 
psychic, 118 
red, 88 

reflex (supposed), 329 
soul, 118 

with acromegaly, 562 
with brain tumor, 391 
with epilepsy, 734 
with lead intoxication, 528 
with migraine, 700 
with multiple neuritis, 602, 604 
with myelitis, 567, 568 
Blind spot, 158 

detection of, 159 
enlargement of, 166 
Brachial plexus, 
diseases of, 592 

ocular signs of, 593 
Brain, anomalies in development of, 545 
Brain tumors, 387 

Benedict symptom complex of, 422 
course and signs of, 387 
dangers of lumbar puncture in, 395 
differential diagnosis of, 432 
lesions simulating, 432 
of cerebellum, 430 

surgical treatment of, 877 
of cerebral peduncle, 421 
of chiasm surgical treatment of, 876 
of corpora quadrigemina of, 403 
of frontal lobe, 395 
of medulla and pons, 429 
of motor area, 399 
of occipital lobe, 387 

surgical treatment, 873 
of optic nerve, 428 
of optic thalamus, 400 
of parietal lobe, 395 
of pituitary body and pineal gland, 42(5 
of temporal lobe, 399 
symptoms of, 387 

resemblance of, to multiple 
sclerosis, 419 
Bulb (medulla oblongata), 

acute apoplectiform paralysis of, 445 
aneurism of, 451 
disease of, 451 

symptoms of, 435 

with typhoid fever and leukaemia, 
451 
embolism, hemorrhage, and thrombosis of, 

445 
meningeal thickening of, 451 
myelitis of, 451 
neuritis of, 451 
progressive palsy of, 436 
softening of, 448 
traumatic affections of, 451 
Bulbar (and pseudo-bulbar) diseases, 435 
general symptomatology of, 435 
paralysis (palsy), 436 

acute apoplectiform, 445 



Campbell's conclusions, 72 
Car sickness 

due to asthenopia, 819 
Catalepsy, 

from eye-strain, 758 



IXDEX 



975 



Cavernous sinus, 

thrombosis of, 868 

diagnosis of, 871 
surgical aspects of, 871 
symptoms of, 870 
Centres (cerebral), 
color, 76 

convergence, 195 
grammatical, 148 
language co-ordinating, 148 
macula, 77 
naming, 150 
of sight, 76 
speech and allied, 135 
visual (primary), 76 

relation of eyes to (diagram), 129 
relation to optic tracts 
(scheme of), 104 
Cerebellum, 

diagnosis of lesions of, (see also Brain tu- 
mors), 424 et seq. 
operations on, 

anatomic considerations, in 878 
indications for, 877 
palliative, 883 
results of, 884 
technique of, 879 
small, resemblance to Friedreich's disease. 

550 
tumors of 

(see Brain tumors) 
Cerebral 

absence, 549 
atrophy, 549 
defects, 549 
iissuration, 

abnormal, 551 
heterotopia, 551 
palsies 

(see Paralysis) 
conjugate, 266 
of children, 538 
sclerosis, diffuse, 474 
surface, 

function of, 72 
Choked disc, 

operations for relief of, 877 
significance of, in brain tumor, 426 
Chorea, 

atrophy of cerebrum with, 519 
facial, 801 
habit spasm, 799 
relation of eye-strain to, 799 
with brain tumor, 400, 421 
with exophthalmic goitre, 830 
Chorioid, 

anatomy of, 24 
Chromatic aberration, 36 
Chromatopsia, 

hysterical, 669 
Chromidrosis, 

hysterical, 669 
Ciliary 
body, 

anatomy of, 23 
muscle, 

hyperesthesia of (hysterical), 640 
nerves, 

origin of, 25 
Clock-face chart, 53 
Cochlear nerve, 

intracerebral anatomy of. 17 
amnesia, anomia, aphasia. 101 
Colorblindness (achromatopsia), 172 
causes and detection of, 1 72 
central. 88 



Color blindness (achromatopsia), hysterical, 101, 
633 

partial. 8S 
uniocular, 102 
with myelitis, 567 
disorders, 

hysterical, 101, 633 
field, 

contraction of, 

with acromegaly, 562 
hysterical, 629, 630 
fundamental 

centres for recognition of, 92 et seq. 

clinics pathologic position of, 94 et 
seq. 
cortical perception of, 90 
Wildbrand's hypothesis of, 92 
impairment, 

focal causes of, 91 
hysterica], 101 
phenomena, 

cerebral, 88 
hysterical, 101, 633 
terms used in study of, 89 
theory of, 90 
recognition, 

cortical centres for, 88 
sensations, 

centre for, 76 

cerebral activity regarding, 89 
hysterical disturbance of, 633, 101 
paths of transmission of, 91 
with ocular arteriosclerosis, 466 
scotomata, 

central, 163 
hysterical, 631 
Conjugate, 

pai'alysis and spasm, 
causes of, 272 
cerebellar. 270 
cerebral, 269 
course of, 266 
diagnosis of, 267 

differential , 267, 272 
nature of, 266 
pontine, 270 

site of lesion causing, 269 
diagnosis of, 271 
symptoms of, 266 
treatment of, 273 
restricted movements, 
non-paralytic, 267 
spasmodic deviation, 
hysterical, 661 
Conjunctiva and cornea, 
anaesthesia of, 322, 

hysterical, 667 
anatomy, 21 
ecchymosis of. 

with epilepsy. 736 
erosions of, 329 
hypenvsthesia of, 

hysterical. 668 
reflex of, 351 
ulcer of, 

dendritic. 329 

with exophthalmic goitre. 839 
Conjunctivitis. 

neurasthenic. 686 
with exophthalmic goitre. s:->9 
Contraction. 

amplitude of, 295 
hysteria! anomalies of. 657 
Convergence, 

loss of. in tabes, 50-4 
movements of. 195 



976 



INDEX 



Convergence, near point of, 206 
nerve centre for, 195 
neurasthenic insufficiency of, 675 
prism, 206 

reaction in brain tumor, 404 
reflex, 196 
tests for, 206 
unilateral, 

with exophthalmic goitre, 836 
Convulsions due to brain tumor, 395, 399 

to cerebral atrophy, 549 
Corpora quadrigemina, diagnosis 
of lesions of, 424 
(see also Brain tumors) 
Corpus callosum, 
absence of, 550 
tumors of (see Brain tumors) 
Cough and phonation, 

difficulties of, in bulbar palsy, 438 
Cranial hernia, 554 
Cranial nerves 

in multiple neuritis, 601 
intracranial portion of, I 
abducens, 16 
accessorius, 19 
acoustic, 17 
cochlear, 17 
facial, 17 

glossopharyngeal, 18 
hypoglossal, 19 
oculomotor, 11 
olfactory, 1 
optic, 1 

pneumogastric, 18 
spinal accessory, 19 
trigeminal 15 
trochlear, 15 
vagus, 18 
vestibular, 18 
nuclei of, 

defective development of, 552 
paralysis of, with acromegaly. 562 

with brain tumor, 393, 421, 429 
with bulbar palsy, 439 
with exophthalmic goitre, 830 
with migraine, 714, 717, 718 
with multiple neuritis, 601, 603, 

604, 605 
with myelitis, 568 
with paresis, 488 
with tabes, 501, 504 
Cranial sinuses, 

lesions of, 867 
Craniectomy, 882 
Cutaneous signs, 

with exophthalmic goitre, 841 
Cycloplegia (see Palsy of accommodation) 
Cycloplegics 

for testing vision, 51 



D 



Dalrymple's sign, 350, 385 
Deafness, 

with brain tumor, 404, 423, 429, 431 

with ocular palsy, 240 

with tabes, 502 
Deafness (mind), 122 
Deafness (word), 

with migraine, 726 
Degeneracy, stigmata of, 951 
etiology of, 960 
list of, "967 
Deglutition and mastication, 

difficulties of, in bulbar palsy, 438 



Dementia, 

with multiple neuritis, 604 
Deorsum version, 268, 269 
Deviation (constitutional), 

biographical study of, 959 

causes of, 961 

in relation to eye-strain, 959, 954 

signs of, 959 
Diabetes, 

visual disorders of, 741 
Digestive disorders, 

due to exophthalmic goitre, 843 

due to eye-strain, 807, 808 

due to migraine, 701 
Dilator pupillae, 286 

sympathetic tract of, 297 
Diplopia, 

artificial or prism, 184 

character of images in, 187 

crossed, 183 

heteronymous, 183 

homonymous, 183 

incongruity of images in, 187 

overcoming, 185 

pathological/ 184 

mechanism of, 184 

physiological, 183 

prism or artificial, 184 

varieties of (table), 186 

with ocular palsy, 209, 213 

with epilepsy, 735 

with migraine, 717 

with tabes, 502 
Dissociated eye movements, 

hysterical, 666 
Divergence, 

anomalies of, 249 

in vertical plane, 196 

movements of, 196 
centres for, 196 
tests for, 207 

paralysis, hysterical, 658 
squint due to, 255 
Double consciousness, 

with migraine, 725 
Dumbness (word), 148 
Dyschromatopsia, 88 

epileptic, 733 

hysterical, 631 
Dyskinesia, 245 
Dyslexia, 139 



| Bmmetropia, 40, 749 
j Encephalitis, superior 

(Wernicke's disease), 

analogy of, to poliomyelitis, 453 
Eneephalocele, 554 
I Enophthalmus, 

with myelitis, 567 
Entropion, 

spasmodic, 337 
Epilepsy, 

definition of, 782 

etiology of, 784 

from ophthalmologic examination, 737 

ocular signs of, 731, 789 

resemblance of, to migraine, 710 

retinal, 734 

sequelae (ocular) of, 737 

simulation of, by hysteria, 637 

treatment of, 795 

with eye-strain, 757, 957 

with multiple neuritis, 604 
Epiphora, 

hysterical, 668 



INDEX 



977 



Epiphora, with exophthalmic goitre, 840 
Erb-Hoppe-Uoldflain, 

symptom complex of, 457 
Erb's disease, 457 

etiology and course of, 461 
symptoms of, 459 
treatment of, 462 
Erythropsia (red vision), 176 
Esophoria, 

asthenopia from, 781 
muscular anomalies, with, 781 
refractive errors, with, 781 
Exophthalmic goitre, 
bibliography of, 849 
corneal changes, with, 839 
course and prognosis of, 843 
cutaneous signs of, 841 
digestive signs of, 843 
exophthalmus, with, 831 
frequency of, 844 
generative signs of, 843 
lachrymation anomalies with, 840 
lid signs of, 833 
nervous signs of, 829 
ocular signs of, 831 

unusual, 840 
pathology of, 844 
respiratory signs of, 841 
treatment of, 845 

surgical, 847 
urinary signs of, 843 
vascular signs of, 828 
Exophthalmus, 

neurasthenic, 687 
surgical treatment of, 847 
with acromegaly, 561 
with goitre, 831 
with myelitis, 568 
Expression (staring), 

with exophthalmic goitre, 835 
External rectus, 

paralysis of, from meningitis, 16 
Eye, aqueous humor of, 30 
arteriosclerosis of, 465 

affections caused by, 468 
associated movements of, 406-408 
ataxia of, 244, 505 
binocular movements of, 193 
check ligaments of, 189 

action of, 190 
conjugate deviations of, 

causes of, 406 
deviations of, 179, 

anomalous, 283 
conjugate, 406 
lateral epileptic, 734 
disjunctive movements of, 283 
dryness of, 

with exophthalmic goitre, 840 
equilibrium of, 143 
examination of, 152 
fundus of (normal), 362 
hysterogenetic zones about, 668 
illumination (oblique) of, 156 
imperfections of, as refractory apparatus, 35 
lateral deviation of 

epileptic, 734 
monocular movements of, 194 

tests for, 204 
movements of, 

anomalies of (tests for), 207 
associated, 

hysterical, 408 

influence of cerebral cortex on, 408 
palsy of, 410 
ataxia of, 244, 505 

62 



Eye, movements of, binocular, 193 
centre for, 406 
lateral (palsy of), 410 
centre for, 406 
transitory palsy of, 407-408 
normal condition of, 207 
physiology of, 178 
searching, 273 
muscles of, 188 
action of, 190 

field of, 193 
anatomy of, 188 
ataxia of, 244 
internal, 285 

and external, 

interdependence of function of, 
778 
operations on, 

for insanit}', 853 

mental disturbances after, 854 

bibliography of, 865 
psychologic effect of, 852 
paralyzed, 

rule to determine, 215 
position of, 

determination of, 154 
equilibrium, 193 
protrusion of, 

see also Exophthalmus, 155 
refractory condition of, 
determination of, 50 
properties of, 31 
surfaces and media of, 34 
relation of (physiologic), 
to refractory errors, 777 
to visual centre, 129 
searching movements of, 273 
sensitive spots about, 

hysterical, 668 
stomach and 

nerve connections between, 808 
strain, 

effects of, 

accommodation spasm, 957 
asthenopia, 814 
catalepsy, 758 
chorea, 

relation of to, 799 
constitutional deviation, 959 
epilepsy, relation of to, 957 
errors of refraction, 748, 749, 760, 

762 
facial twitching, 763 
gastric disturbance, S07, 808 
headache, 743 et seq. 
and insomnia. 7H4 
character of, 955 
location of, 752 
mechanism of, 773 
treatment of, 767 et seq. 
head-bowing of infants, 825 
hypochondriasis, 956 
insanity. 7S0 
malaise and fatigue, 762 
mental attitude in, 95S 
migraine. 71 1 

nerve exhaustion and irritability, 
776. 955 

character of. 956 
visual apparatus in. 77i 
ocular neurosis. 758 
ocular palsies, 822 
physical characteristics, 959 
poor progress at school, 7t :> - 
pseudotorticollis, 823 
psychopathies, 956 



978 



INDEX 



Eye strain, effects of, restlessness, 763 

spasm of accommodation, 957 

of head, 821 
spinal curvature, 823 
torticollis, 823 
vertigo, 803 

diagnosis of,, 804 
treatment of, 805 
structure of, 20 

sympathetic nerves of, 338 
diseases of, 340 
tremor of, 273 

lesions causing, 280 
vascular lesions (epileptic) of, 736 
vasomotor disorders of, 339 
visual power of, 

determination of, 50 



Facial 

anaesthesia, 321 
hemiatrophy (progressive), 556 
causes and pathology, 556 
diagnosis and symptoms, 557 
hemihypertrophy, 558 
nerve, 

clinical anatomy of, 332 
intracerebral anatomy of, 17 
paralysis of, 333 
etiology of, 335 
symptoms of, 333 
treatment of, 335 
surgical, 885 

results of, 891 
with abducens paralysis, 16 
with multiple neuritis, 602 
with myelitis, 568 
with ocular palsy, 240 
peripheral lesions of, 332 
spasm, 335 
twitching, 

due to eye-strain, 763 
Fixation, 

binocular, 

derangements of, 179 
field of vision in, 168 
test for, 205 
squint, caused by, 187 
central, 

mechanism of, 178 
eccentric, 

mechanism of, 178 
field of, 

mechanism of, 192 
tests for, 204 
monocular, 

mechanism of, 178 
tests for, 197 
Foot-drop, 

with multiple neuritis, 594 
Friedreich's disease, 475 
diagnosis of, 475 
gait in, 946 
ocular signs of, 474 
Functional insanity (see Insanity), 508 
Fundus changes 

with exophthalmic goitre, 841 
Fusion faculty, 
centre of, 182 



G 



Gait, 



ataxic, 944 
chicken, 943 



Gait, classification of, 940 
flaccid paralytic, 941 
in anterior poliomyelitis, 941 
in astasia abasia, 948 
in ataxia (locomotor), 944 
cerebellar, 946 
cerebral, 947 
in athetosis, 947 
in brain tumor, 400 
in cerebellar disease, 946 
in cerebral disease, 946 
in chorea, 947 
in Friedreich's disease, 946 
in hemiplegia, 943 
in hysteria, 947 
in lateral sclerosis, 943 
in Little's disease, 943 
in locomotor ataxia, 944 
in multiple neuritis, 947 
in muscular dystrophy, 942 
in myotonia congenita, 948 
in paralysis 

agitans, 948 
flaccid, 941 
spastic, 943 
in peripheral neuritis, 941 
in pseudomuscular hypertrophy, 942 
in sciatica, 949 
in spastic paralysis, 943 
in tabes dorsalis, 944 
in Thomsen's disease, 948 
method of study of, 938 
symptomatic, 937 
varieties of, 940 
Gasserian ganglion, 

division of sensory root of, 900 

advantages of, 901 
technique of, 901 
excision of, 902 

technique of, 903 
physiologic extirpation of, 900 
Gastric disturbances 

due to eye-strain, 807 

mechanism of, 808 
due to migraine, 701 
Generative disorders, 

with exophthalmic goitre, 843 
Gerlier's disease, 465 
Glaucoma, 

sympathectomy for, 341 
Glossopharyngeal nerve, 

intracerebral anatomy of, 18 
Graefe's sign, 350, 833 



H 

Harlan's test, 693 
Head 

bowing of infants, 825 
epileptic deviation of, 734 
movements (spasmodic). 
causes of, 821 
classification of, 824 
Headache 

and insomnia from eye-strain, 764 
blind, 

in migraine, 706 
with asthenopia, 816 
with exophthalmic goitre, 830 
with eye-strain, 743 et seq. 
location of, 752 
mechanism of, 773 
treatment of, 767 
with migraine, 706 
with ocular palsy, 211 
with refractive errors, 748 



INDEX 



979 



Hemeralopsia 

(day blindness), 175 
Hemiachromatopsia, 88 
Hemialencopsia, 87 
Hemianesthesia, 

visual disturbances in, 114 
Hemianopsia (hemianopia), 

cortical, 79 

determination of, 86 
definition of terms used, 80, 428 
differentiation of, 85 
relative, 84 

forms of, 87 

heteronymous. 167 

homonymous, 

causes of, 167 

hysterical, 634 

incomplete, 

causes of, 168 

location of lesions in, 81 et seq. 

subcortical, 86 

temporal (causes of), 170 

with acromegaly 562 

with brain disease, 426 

with lead intoxication, 529 

with migraine, 700 
Hemianopic hallucinations with migraine, 708 

pupilreflex, 318 
Hemiasynergy, 431 
Hemichromatopsia, 168 

amblyopia with, 169 

functional or hysterical, 170 

visual field defects in, 169 

lesions causing, 169 
Hemicrania (migraine), 701 
Hemiplegia, 

due to atrophy of cerebrum, 549 

position of head after, 822 
Hereditary cerebellar ataxia, 475 
Heterophoria 

(see Squint), 180 
Hippus, 674, 316 
Hydrencephalocele, 

diagnosis of, 554 
Hydrocephalus, 

congenital, 551 
signs of, 552 
Hyperopia 

(hypermetropia), 40 

absolute, 42 

axial, 41 

causes of 40 

curvature, 40 

facultative, 42 

in childhood, 42 

index, 41 

latent, 42 

manifest, 42 

total, 42 

treatment of, 43 
Hypertrophy of 

half of body, 558 

of internal organs, 558 

of limbs, 558 
Hypnotism and sleep, 

ocular manifestations of, 689 
Hypochondriasis 

from eye-strain, 956 

ocular manifestations of 688 
Hypoglossal nerve, 19 

palsy of, with myelitis, 568 

with ocular palsy, 240 
Hysteria, 

color fields in, 629 

ocular signs of, 614 

simulation of, to epilepsy, 637 



Hysteria, simulation of, to exophthalmic goitre, 
637 

to insular sclerosis, 637 

to migraine, 722 

to retrobulbar neuritis, 637 

to syndrome of Webster, 653 

to syringomyelia, 637 
treatment of ocular disorders of, 687 
with exophthalmic goitre, 830 
with migraine, 709 
Hysterical 

accommodation anomalies, 641, 645 
achromatopsia, 631 
alopecia of lids, 670 
amblyopia, 669 
anaesthesia of cornea, 667 
associated movement palsy, 665 
asthenopia, 640, 659 
attacks, 

pupils in, 649 
blephai-ospasm, 651 
chromidrosis, 669 
conjugate deviation, 661 
convergence, 657 
color, fields, 629 

scotomata 631 

sense, 632 
cycloplegia, 644 
cyclospasm, 641 
divergence paralysis, 658 
dissociated eye movements, 666 
dyschromatopsia, 631 
epiphora, 668 
hemianopia, 634 
hyperaesthesia of ciliary muscle, 640 

of cornea and conjunctiva, 66S 

of spots about eyes, 668 
insular sclerosis, 637 
iridochoroiditis, 669 
kopiopia, 640 
light and color sense, 

importance of, 632 
migraine, 641 
mydriasis, 647, 649 
myosis, 646, 649 
neurasthenia, 672 
neuroses (traumatic), 672 
nystagmus, 666 
ocular palsies, 663 

stigmata, 670 
opacity of lens, 669 
ophthalmoplegia interna, 648 
painful visual sensations. 640 
paradox pupil reaction, 650 
paralysis of accommodation. 644 

of levator, 655 

of orbicularis, 654 
ptosis, 652 

morning and night. 656 
pupillary phenomena, 646 

'during attacks. 649 
retinal changes, 669 
sensitive spots about eye, 668 
spasm (ocular). 654 
strabismus, 659 
traumatic neuroses. 672 
triplopia. 643 
visual field, 633 

sensations. 640 
zones about eye. 668 

I 

Imbecility 

with cerebral atrophy. 549 
Inferior dental nerve. 

exposure of. 899 



980 



IKDEX 



Infraorbital nerve, 

exposure of, 898 
Insanity caused by eye-strain, 780 
functional, 

ocular signs of, 508 
summary of, 524 
relief of, 

operations on eye for, 853 
with exophthalmic goitre, 830 
Irido choroiditis 

hysterica], 669 
IridoJegia, 
reflex, 

causes of, 589 
Iris, 

anatomy of, 22, 285 
examination of, 288 
function of, 287 
method of measuring, 289 
rigidity of, 

with acromegaly, 562 



K 

Kataphoria, 782 
Keratitis 

exposure, 353, 326 
lagophthalmic, 353, 326 
neuropathic, 325 

course and treatment of, 326 
xerotica 

with exophthalmic goitre, 840 
Keratometry, 55 
Knee-jerk, 925 

with brain tumor, 419, 432 
Kopiopia, 

hysterical, 640 
Korsakoff's psychosis, 595 

with alcoholic neuritis, 595 



Lachrymation, anomalies of: diminished, 344 
increased, 334 
mechanism of, 344 
with exophthalmic goitre, 840 
with tabes, 506 
Lagophthalmos 

from exposure, 352 
from facial paralysis, 352 
from old age, 352 
keratitis with, 326, 353 
Lamina vitrea, 286 
Landry's paralysis 

(see Paralysis, acute ascending), 590 
Lateri version, 267, 268 
Lead intoxication, 

ocular signs of, 528-531 
Lens, crystalline, 

hysteric opacity of, 669 
structure of, 30 
Letter blindness, 137 
Levator spasm and paralysis, 

hysterical (see Hysterical ptosis), 654, 655 
differential diagnosis of, 656 
Lids, alopecia (hysteric) of, 670 
examination of, 153 
movements of, 192, 345 

harmony of, with those of eyeball, 
348 
oederna of, 

with exophthalmic goitre, 840 
reactions of, 306 
reflexes referable to. 351 
signs referable to, 350, 351 

with exophthalmic goitre, 833 



Lids, tremor of, 

with exophthalmic goitre, 840 
Lid signs : Dalrymple's sign, 350 
Graefe's sign, 350, 351 
of goitre, 833 
Stellwag's sign, 351 
Light reflexes, 290 

arc of, course of fibres in, 290 et seq. 
lesions of, 291, 292, 294 
examination of, 299 
Light test, 

Wernicke's or Wilbrand's, 170 

mechanism, significance and value 

of, 170 
methods to apply, 171 
Locomotor ataxia 

(see Tabes dorsalis) 
Lumbar puncture, 

danger of, in brain tumors, 395 



M 

Macropsia, 

with epilepsy, 733 
Macula lutea, 

anatomy of, 29 
functions of, 108 
Macular bundle, 

distribution of fibres of, 109 
centre, connecting tracts of, 111 
fibres, 

decussation of, 4 
representation centre, 103 
location of, 110 
Maddox rod test, 201 
Mastication and deglutition, 
difficulties of, 

in brain tumor, 429 
in bulbar palsy, 438 
Medulla and pons, 
tumors of, 429 
Membrane of Baruch, 286 
Meningocele, 554 
Mental alterations, 
(see Psychoses) 
attitude, 

anomalies of, due to eye-strain, 958 
Microcephalus, 547 
diagnosis of, 548 
treatment of, 549 
Microphthalmus, 552 
Micropsia, 

with epilepsy, 733 
Migraine, 

auraa with, 700 

causes of, 698 

eye-strain in relation to, 711 

gastric disorders of, 701 

hemicrania with, 701 

hysterical ophthalmic, 641 

in hysteria, paresis, and tabes, 709 

malaria as a cause of, 721 

motor type of, 702 

nasal catarrh in relation to, 721 

nuclear ophthalmoplegia differentiated 

from, 720 
ophthalmic, 703 

aphasia with, 708 
headache in, 706 
hysterical, 641 
visual signs of, 703 et seq. 
ophthalmique accompagnee, 708 
ophthalmoplegic, 243, 712 
course of, 713 

cranial nerve palsies with, 714, 717, 718 
diagnosis of, 719 






INDEX 



981 



Migraine, ophthalmoplegic, pain and paralysis 
in, 713, 714 

optic illusions with, 700 

pain of, 701 

pathology of, 710 

psychical, 702, 723 
symptoms of, 725 

relation to epilepsy, 711 

resemblance to epilepsy, 710 

scotoma with, 175 

spectra of, 114 

symptoms of, 699 

tabes in relation to, 721 

treatment of, 726 

varieties of, 697 

with exophthalmic goitre, 830 
Mind-blindness, 

causes of, 122, 127 

synonyms for, 118 
with brain tumor, 398 
Mobius sign, 836, 932 
Multiple sclerosis, 

ocular signs of, 469 

resemblance of, to brain tumor, 419 
Muscular atrophy, 

with exophthalmic goitre, 830 

contraction, 932 

difference of, from reflexes, 910 
idiopathic, 932 
Myasthenia gravis, 457 
Myasthenic reaction, 

with Erb's disease, 462 
Mydriasis, 

hysterical (see Dilatation of pupils), 647 

interchange of myosis and (hysterical), 649 

spastic, 314 
Myelitis, 565 

cranial nerve palsies with, 568 

etiology of, 565 

histology of, 569 

pathology of, 565 

prognosis and treatment of, 572 

relation of, to optic neuritis, 571 

symptoms of, 565 

treatment of, 572 
Myoidema, 932 
Myopia, 

apparent, 52 

axial, 43 

benign or school, 45 

curvature, 43 

index, 43 

malignant, 45 

school, 45 

treatment, 45 

with hysterical cyclospasm, 642 
Myosis, hysterical, 646 

interchange of, with mydriasis, 649 



N 

Nasal smile with Erb's disease, 460 
Near-sightedness 

(see Myopia), 43 
Nervous irritability and exhaustion 

due to eye-strain, 776 

character of, 956 
visual apparatus in, 776 

with exophthalmic goitre, 829 
Neuralgia, 

facial 

(see Trigerminal neuralgia) 
Neurasthenia, 

effect of asthenopia on, 779 

hysteric manifestations of, 672 

Rosenbach's sign of, 674 



Neuritis, 

multiple, 

alcoholic, 549 

arsenical, 603 

beriberi, 608 

carbon monoxide and disulphide, 603, 
604 

carcinomatous, 611 

diabetic, 613 

diagnosis of, 534, 600 

diphtheritic, 605 

etiology of, 594 

influenzal, 608 

lead (plumbic), 600 

leprous, 609 

malarial, 608 

pathology of, 598, 601, 607 

senile, 610 

sulphonal and trional, 604 

symptoms of, 594 

syphilitic, 609 

tuberculous, 609 
retrobulbar, 

simulation of, by hysteria, 637 
Neuroretinitis due to lead intoxication, 529 
Neuroses, ocular, 

due to eye-strain, 758 
traumatic, 

hysteric manifestations of, 672 
Neurotabes, 594 
Numeral-blindness, 142, 
Nyctalopia 

(night-blindness), 176 
Nystagmus, 273 
atactic, 474 
epileptic, 735 
etiology of, 276 
hysterical, 666 
occupational, 277 
pseudo, 273, 474 

etiology of, 277 
symptoms of, 281 
treatment of, 282 
unilateral, 275 
variations in, 275 
varieties of, 274 
voluntary, 279 
with acromegaly 562 
with ataxia, 474 
with brain tumor. 432, 417 
with epilepsy, 735 
with exophthalmic goitre, 840, 841 
with Friedreich's disease, 474 
with multiple neuritis, 600, 603 
with myelitis. 568 



Occipital lobe, 
tumors of 

(see Brain tumors), 873 
Ocular 

defects (hysterical), 614 
deviation, 250 

measurement of, 198 
equilibrium, 

position of, 193 
innervation, 

impairment of, due to lead, 528 
movements, 
binocular, 

parallel, 193 
tost for. 205 
centres for. 195 
etiologic classification of, 208 
incoordination of 

(diplopia with). 195 



982 



INDEX 



Ocular movements, tests for, 196 

muscles, anomalies, neurasthenic, of, 674 
associated action of, 193 
central nerve, supply of, 12 et seq. 
epilepsy of, 134 
neurasthenic anomalies of, 674 
paralysis of, 209 

associated palsies with, 239 

basal, 228 

causes of, 234 

conditions allied to, 245 

cortical, 221 

course and prognosis of, 241 

diagnosis of, 211 

differential, from spasm, 247 
of cause of, 234 
of site of lesion in, 237 
value of associated paralysis 
in, 239 

due to acromegaly, 233, 562 
to epilepsy, 737 

to alcohol and other poisons, 234 
to autointoxication, 234 
to basal brain lesions, 220, 238, 

227 
to beriberi, 233 
to brain tumors 400, 403, 405, 

421, 429 
to cerebro spinal fever, 233 
to congenital conditions, 222 
to diabetes, 233 
to diphtheria, 231 
to erysipelas, 233 
to exophthalmic goitre, 233, 

836 
to extracranial lesions, 219 
to general paresis, 229 
to Gerlier's disease, 233 
to hemorrhage, 234 
to heredity, 235 
to herpes zoster, 232 
to hysteria and allied neuroses, 

230 
to influenza, 231 
to intracranial lesions, 220 
to kidney disease, 233 
to malaria, 233 
to meningitis, 227 
to multiple neuritis, 603 etseq. 
to mumps, 233 
to nasal affections, 234 
to nuclear disease, 239 
to orbital disease, 237 
to pneumonia, 233 
to pontine disease, 239 
to pyaBmia, 233 
to renal diseases, 233 
to rheumatism, 232 
to sclerosis, 229 
to spinal disease, 228 
to syphilis, 230 
to tabes, 228, 501 

frequency of, 504 
to tetanus, 233 
to traumatism, 225 
to tuberculosis, 232 
to typhoid, 232 
to Weil's disease, 233 
to whooping cough, 233 

etiology of, 218 

exciting causes of, 222 

fascicular, 220 

hysterical, 230 

innervational, 219 

insertional, 218 

intracranial, 220 



Ocular muscles, paralysis of, nuclear, 220, 239 
oculomotor, 235, 237 

with brain tumor, 404, 421 
orbital, 219, 237 
peripheral, 219 
pontine, 239 
prognosis of, 241 
significance of associated, 405, 415, 

417 
structural, 218 
supranuclear, 221 
symptoms of, 209 
(see Ophthalmoplegia) 
treatment of, 244 
total, 218 
varieties of, 217 
spasm of, 246 

etiology of, 248 
evidence of, 246 
primary, 

affections characterized by, 248 
resemblance to paralysis, 247 
secondary, 

affections characterized by, 249 
true, 248 
nerves, 

tumors of, 387 
neuralgia, 

with acromegaly, 562 
with myelitis, 567 
neuroses, 

with eye-strain, 758 
oedema, 324 

pain, significance of, 323 
palsies, 

following epilepsy, 737 
reflex (supposed), 329 
with acromegaly, 562 
with basal meningitis, 12 
with Friederich's disease, 476 
with hysteria, 663 
with lead intoxication, 531 
with migraine, 714, 717 
with multiple neuritis, 595, 603, 608 
stigmata, hysterical, 614, 670 

etiology of, 670 
symptoms, 

relation to spinal disease, 579 
with exophthalmic goitre, 831, 840 
with hysteria, 614 
Oculomotor nerve, 

intracerebral course of, 11 
nucleus of, 11 

basal co-ordinating centre of, 412 
function of, 411 
subdivisions of, 13 
paralysis of, 214, 218 
Oculopupillary, 

fibres, course of, 588 
reflex, 304 
Olfactory palsy with ocular palsy, 239 
Ophthalmic examination, 

epileptic seizure caused by, 737 
herpes, 326 
signs of disease, 364 
Ophthalmoplegia, 
chronic, 454 

progressive, 242 
hysterical, 648, 665 
interna, 312 
nuclear, 720 
total, 218 

with brain tumor, 404, 419 
with migraine, 719 et seq. 
diagnosis of, 719 
with myelitis, 568 



INDEX 



983 



Ophthalmoplegia, with ocular palsy, 237 

with tabes, 498, 503 
Ophthalmoscopy, 54, 360 
instruments for, 360 
technique of, 361 
Optic, 

aphasia, 

centre affected in, 150 
lesions causing, 149, 150 
mechanism of, 149 
atrophy, 

ascending, consecutive or neuritic, 384 
ophthalmic appearances in, 382, 384 
prognosis and treatment of, 3S4 
symptoms of, 383, 385 
with acromegaly, 562 
with myelitis, 567, 569 
with paresis, 480 
with pituitary diseases, 426 
with tabes dorsalis, 499 
centres, 

primary, 5 
chiasm, 

decussation of fibres in, 1 
structure of, 3 
surgical treatment of. 876 
tumoi-s of, 876 
illusions, 

with migraine, 700, 707 
nerve, 

anatomy of, 1 
atrophy of, 

due to acromegaly, 562 
to arteriosclerosis, 467 
to lead intoxication, 529 
to sclerosis, 469 
hereditary, 385 
primary, 382 
secondary, 384 
hemorrhage into, 368 
inflammation of 

(see Optic neuritis), 375 
involvement of, in spinal diseases, 585 
multiple sclerosis of, 469 
palsy of, 

with ocular palsy, 239 
tumors of, 428 

early failure of vision, 429 
hallucinations of sight in, 429 
reflex pupillary response in, 429 
neuritis, 375 

acute retrobulbar, 380 
causes of, 375 
chronic retrobulbar 

(toxic amblyopia), 381 

ophthalmic appearances in, 382 
pathology and symptoms of, 

381 
prognosis of, 382 
color field in. 378 
mechanism of intracranial, 376 
ophthalmic signs of, 377 
prognosis of, 379 
relation of, to myelitis, 571 
symptoms of, 377 
treatment of, 380 
visual acuity in, 377 
visual field in, 378 
with brain tumor, 430, 419 
with Friedreich's disease, 476 
with lead intoxication, 529 
with multiple neuritis, 602, 576, 598 
with myelitis, 566 
with pituitary disease, 426 
tracts, 

intracerebral course of. 5 



Optic tracts, visual centres, scheme of, 104 
Orbicularis, 

paralysis, hysterical. 654 
reaction, 306 
Orbit, 

relation of structures of, 347 
Orbital pain, 

with acromegaly, 562 
with myelitis, 567 
with tabes, 505 
Orbito palpebral sulcus, 
obliteration of, 

with exophthalmic goitre, 840 
Orientation, 

lesions causing, loss of, 84 
Orthophoria, 180 



Palpebral opening, 

changes in form of, 349 

variations in size of, 348 
widening of, 

due to contraction of elevator, 352 
Palsies, 

acute bulbar, apoplectiform, 445 
bulbar, progressive, 436 
course of, 445 
electrical reactions in, 441 
etiology of, 444 
historical data of, 436 
pathology of, 442 
physical signs of, 439 
pseudo, 454 
symptoms of, 436 
accessory, 439 
general, 435 
synonyms for, 436 
treatment of, 445 
cerebral, of children, 539 

differential diagnosis of, 543 
etiology of. 539 
eye symptoms of, 542 
pathology of 542 
symptoms of, 539 

ocular, 542 
treatment of, 544 
types of, 538 
Parachromatopsia, 88 
Parallax test, 200 
Parallel movements 
(associated), 
hysteric palsy of, 665 
Paralysis, 

acute ascending. Landry's, 590 
etiology of, 591 
symptoms of, 590 
ocular. 591 
agitaus. 476-477 
asthenic, 457 
cerebral of children 

(see Palsies), 538 
conjugate, 266 
extra-ocular. 

with exophthalmic goitre, 836 
of associated parallel movements, hysteric, 

665 
unilateral progressive 

ascending spinal, 581 
with exophthalmic goitre, 830 
Paresis. 

juvenile, 491 

ocular signs of, 478 et seq. 
physical signs of. 488 
sequence of phenomena in. 189 
summary of ocular si;in< o\\ -188 
with migraine, 7(10 



984 



INDEX 



Perimeter 

for testing fixation, 204 

portable, 159 
Pernicious anaemia, 

spinal signs of, 582 

ocular signs of, 583 
Phonation and cough, difficult 

with bulbar palsy, 438 
Phorometer, 202 
Photophobia, 

significance of, 330 

treatment of, 331 

with epilepsy, 733 
Physical peculiarities 

due to eye-strain, 959 
Pineal gland, 

diagnosis of lesions of, 426 
Pituitary body, 

diagnosis of lesions of, 426 
Pneumogastric nerve, 

intracerebral anatomy of, 18 
Polioencephalitis, acute, 

diagnosis of, 

from multiple neuritis, 600 
Poliomyelitis, ' 

anology of, to encephalitis superior, 452 
Polyneuritis 

(see Multiple neuritis) 
Pons, 

embolism, hemorrhage and thrombosis of, 
445 

anatomic facts in, 445 
pathology of, 446 
symptoms of, 447 

softening of, 448 

course, prognosis, and treatment of, 450 

tumors of, 429 
Porencephaly, 

causes and types of, 546 

diagram of, 547 
Position sense, 

cortical centre of, 397 
Posterior lateral sclerosis, 581 
Presbyopia, 39 
Projection, binocular, 182 

monocular, 178 
law of, 179 
Proptosis, 

determination of, 155 

with exophthalmic goitre, 830 
Pseudobulbar paralysis, 454 
pathology of, 455 
prognosis and treatment of, 457 
symptoms of, 456 
Pseudokinesis, 210 
Pseudosclerosis of Westphal, 479 
Pseudoscopic apparatus, 694 
Pseudotabes, 594 

Pseudotorticollis due to eye-strain, 823 
Psychic blindness, 118 
Psychopathies 

due to eye-strain, 956 
Psychoses 

after eye operations, 854 

bibliography of, 865 
etiology of, 858 
groups of, 857 
treatment of, 864 

Korsohoff's, 595 

with brain tumors, 398 et seq. 

with exophthalmic goitre 829, 830 

with migraine, 725 

with multiple neuritis, 601, 604 
Ptosis, atonic, 355 

general characteristics of, 353 

hysteric, 656 



Ptosis, neurasthenic, 675 
pseudoparalytic, 652 
spastic, 653 
traumatic, 356 
with brain tumor, 416 
with multiple neuritis, 602, 604, 608 
with myelitis, 568 
with ocular palsy, 236 
with paresis, 488 
with tabes, 502 
Puerperal eclampsia, 

eye symptoms of, 740 
Pupil, 

Argyll Robertson, 308 

frequency and significance of, 493 
lesions causing, 309 
unilateral, 495 
dilated, 

with multiple neuritis, 603 

with paresis, 482 

with tabes, 497, 498 
during gastric crises, 498 
fixation of, 

with acromegaly, 562 

with multiple neuritis, 603 
inequality of, 674 

with brain tumor, 404 

with multiple neuritis, 604 

with paresis, 479, 481 

with tabes, 496, 498 
in hysteric attacks, 649 
light reaction of, 315, 604 
narrowed, 

with multiple neuritis, 603 

with paresis, 480 

with tabes, 496 
neurasthenic signs in, 674 
normal, 288 
paradox, 
in tabes, 505 

pathologic variations of, 307 
phenomena of, 

with epilepsy, 735, 736 

with myelitis, 567 
rigidity of, 

with multiple neuritis, 603 
Pupillary 

centre (reflex), 589 

dilator, 286 

lid reaction, 306 

nerve-fibres, 

anatomy and physiology of, 5 
orbicularis reaction, 306 
phenomena, 

epileptic, 735 

medicolegal value of, 736 

hysterical, 646 
reflexes, 290 

convergence, 295 

hemianopic, 318 

light, 290 

Marian's experiments, with 296 

myotonic, 314 

oculo, 304 

on movement of eyes, 307 

pain, 303 

paradoxical, 317 
hysterial, 650 
sphincter, 285 

R 

Red-glass test, 200 
Reflexes, 

abdominal, 930 

Achilles-tendon, 925 

adductor, 925 

ankle-clonus, 926 



INDEX 



985 



Reflexes, attention, 304 
Babinsky's, 927 

modifications of, 923 
bicipital, 918 
chin-jerk, 917 
conditions increasing, 915 
conjunctival (corneal), 351 
cortex, 305 
Chvostek's, 916 
cremasteric, 931 
definition of, 910 
differences of, from muscular contraction, 

910 
femoral, 929 
frontal, 930 
fundus, 362 
gluteal, 932 
humeroscapular, 932 
hypogastric, 931 
hypothenar. 919 
iliac, 932 
Kernig's, 929 

knee-jerk, patellar. 919, 924 
method to elicit, 920 
significance of. 911, 922 
lid, 351 
light, 290 

arc of, 290 

fibres of, 291, 294 

decussation of, 292 
lesions of. 291, 292, 294 

examination of 299 et seq. 
literature on, 933 
malar. 917 
mechanical, 910 
mechanism of, 911 
method of eliciting, 915 
nature of, 914 
patellar, 924 

clonus, 922 

with brain tumor, 419, 432 
plantar, 927 
pupillary cortical, 304 
psychical, 910 
pyramidalis, 931 
scapulohumeral, 932 
Sinkler's toe-jerk, 930 
study of, 915 
supinator, 919 
supra-orbital, 351, 916 

significance of, 916 
tibialis phenomenon, 930 
tricipital, 919 
Refraction, 

anomalies of, ammetropia, 40 
effect of asthenopia on, 781 
errors of, 

headache in relation to, 748 

ocular defects and 

position of head in, 822 

physiologic relation of eye to. 777 

with exophthalmic goitre, 841 
Respiratory signs, 

with exophthalmic goitre, 842 
Retina, 

anaemia of, 365 
anaesthesia of, 365 
anatomy of, 26 
angeoid streaks of, 367 
apoplexy of, 367 
arteriosclerosis of. 466 
artery, central, of. 
embolism and thrombosis of. 368 
blindness due to. 742 
causes of, 368 
treatment of, 369 



Retina, blood supply of. 29 

corresponding points of, 182 

diseases of, 360 

dysesthesia of, 

with epilepsy, 733 

epilepsy of. 734 

fatigue of, 160 

function of, 10S 

hemorrhage of, 367 

hyperajsthesia of, 365 
hysterical. 640 

hysteric changes in. 669 

inflammation of, 

(see Retinitis). 370 

macula lutea. 364 

cedema of, 366 

papilla? of. 363 

sensory affections of, 365 

vascularity of, 367 

vascular lesions of, 365 

vein, central, of 

thrombosis of, 369 

vessels of. 363. 29 

anomalies of. 366 
inflammation of. 367 
Retinitis, 370 

albuminuric. 373 

anaemic, 374 

diabetic, 374 

pigmentation, 371 

proliferating. 367 

syphilitic. 372 

varieties of. 371 

with brain tumor. 431 

with paresis. 486 



Sclera, 

anatomy of, 22 
Sclerosis, 

diffuse cerebral. 474 
insular, 

simulation of hysteria to, 637 
lateral. 

amyotrophic, 580 
postero, 5S1 
primary, 5S0 
Scotoma, definition of. 15S 
fatigue. 

neurasthenic. 680 
lead, 529 
ring. 166 

with arteriosclerosis of eye. 466 
with epilepsy. 732 
with migraine. 700. 704. 706 
with multiple neuritis. 603 
Scotoma, annular 
central. 165 
hysteric, 627 
Sight, 

hallucination of. 

following epilepsy. 737 
Skiascopy. 54 

anomalies in development of, 545 554 
with opticatrophy, 554. oo^ 
Sleep and hypnotism, 

ocular manifestations of, 689 
Soul blindness, 1 18 
Speech. 

cerebral zone of, 1 15 

anatomy and physiology of. 115 
factors determining location of, \">j 
disorders of (see also Aphasia), 
with migraine, 925 
with brain tumor. 399 
with bulbar palsy. 437 



986 



INDEX 



Sphincter iris muscle, 293 
Spinal cord, 

diseases and injury of, 583 
gunshot, 588 
ocular signs of, 584 
optic nerve involvement in, 585 
sympathetic disease with, 586, 588 
curvature 

due to eye-strain, 823 
Squint (strabismus), 
alternating, 199 
bilateral nature of, 261 
binocular vision in, 187 
classification of, 180 
convergent, 255 
determination of, 198 
development of, 

conditions favoring, 260 

due to convergence excess, 252 

to convergence insufficiency, 256 
to convergence paralysis, 257 
to divergence paralysis, 255 
to epilepsy, 735 
to esophosia, 252 
to exophoria, 256 
rationale of, 259 
divergent, 258 
epileptic, 735 
hysterical, 659 
nature of, 249 
position of head in, 823 
symptoms of, 263 
tests for, 198 
treatment of, 264 
vertical, 

hyperphoria with, 262 
Stanley Barnes, diagram by, 141 
Stellwag's sign, 351, 835 
Stereognosis, 

cortical centre of, 397 
Stereoscopic test, 204 
Stomach disturbances 

due to eye-strain, 807 
Strabismus 

(see Squint) 
Supraorbital nerve, 

exposure of, 898 
Sursumversion, 268, 269 
Sympathetic nerve, 

involvement of, in spinal disease, 586 

symptoms of, 588 
removal of, for glaucoma, 341 
Syntax, 146 
Syringomyelia, 572 

cranial nerve paralysis with, 578 
ocular palsies with, 576 
pathology of, 573 
symptoms of, 572 et seq. 

ocular, 573 
simulation of, by hysteria, 637 
visual field restriction, 575 



Tabes dorsalis, deafness with, 502 

diagnosis of, from multiple neuritis, 594 
from pseudotabes, 594 
facial anaesthesia with, 505 
gait in, 944 
lachrymation in, 506 

neuralgia (periorbital) in, 505 
ocular crises with, 505 
signs of, 493 

summary of, 506 
pseudotabes, diagnosis of, from true 
tabes, 594 



Tabes dorsalis, lachrymation in, exophthalmic 
goitre, with, 830 

with migraine, 709 
Taste impairment with brain lesions, 430 
Tears, 

secretion of, 

in disease of fifth nerve, 342 
in disease of seventh nerve, 343 
mechanism of, 342 

part played by seventh nerve in, 
343 
variations in amount of, 344 
Tenon's capsule, 

anatomy of, 188 
Test-letters, 50, 51 
Tetanus, 

cephalic, 728 

pathology of, 730 
hydrophobic, 728 
Tetany, 

ocular signs of, 730 
with exophthalmic goitre, S30 
Thyroid enlargement 

in exophthalmic goitre, 830 
Tic douleureux 

(see Trigeminal Neuralgia) 
Tongue, impaired mobility of, 
with brain tumor, 429 
with bulbar palsy, 437, 439 
Torticollis 

due to eye-strain, 823 
pseudo, 823 
with ocular pals} r , 211 
Tremors, 

causes of, 906 
classification of, 907 
congenital, 907 
definition, 906 
diagnosis of, 907 
fibrillary, 429 
imitation, 909 
in brain tumor, 421 
in exophthalmic goitre, 908, 829 
in hysteria, 908 
in multiple sclerosis, 908 
in neurasthenia, 909 
in paralysis agitans, 908 
in paresis, 909 
pseudo, 906 
significance of, 906 
Trigeminal nerve, anaesthesia of, 321 

with ocular palsy, 240 
with tabes, 505 
anatomy of, 320 
distribution of, 321 
functions of, 321 
herpes zoster of, 326 
causes of, 328 

diagnosis from erysipelas, 327 
symptoms and ocular signs of, 317 
treatment of, 328 
implication of, in spinal lesions, 16 
lesions of, 320 
nuclei of, 15 

neuralgia of, (tic douleureux), 323, 894 
medical treatment of, 896, 
surgical treatment of, 897 
neuritis of, 323 
palsy of, 

with ocular palsy, 240 
paraesthesia of, 322 
physiology of, 321 
sensory fibers of, 15 
trophic disorders of, 324 
Triplopia, 

hysterical, 643 



INDEX 



987 



Trochlear nerve, 

intracerebral anatomy of, 15 
Tropometer, 

for testing fixation, 204 

u 

Unconsciousness 

with multiple neuritis, 604 
Uraemia, 

blindness due to, 738 
Urinary disorders, 

with exophthalmic goitre, 843 
Uvea, 

anatomy of, 22 

blood and nerve supply of, 25 



Vagus (pneumogastric) nerve, 
disease of, 

with multiple neuritis, 595 
intracerebral anatomy of, 18 
Vascular disorders, 

with exophthalmic goitre, 828 
Vertigo, 

ocular. 803 

diagnosis and treatment of, 804 
paralyzing 

(Glerlier's disease), 462 
reflex, 803 

with brain tumor, 431 
with migraine, 726 
with ocular palsy, 210 
Vertigo and false projection, 

with conjugate paralysis and spasm, 266 
Vestibular nerve, 

intracerebral anatomy of, 18 
Vibration sensation, 133 
Vision, 

acuteness of, 50 
apparatus of, 

congenital absence of, 552 
retino-intracranial, 65 
tumors in relation to, 367 
binocular, single, 181 

comparison of, to binocular fixation, 187 
in squint, 187 

test for field of, 187, 205, 206, 195, 199 
centre of, and optic tracts, 

scheme of, 104 
cortical area of, 8, 59, 60, 75 

comparison of, in man and lower ani- 
mals, 63 
focal disturbances of, 79 
psychic and sensory areas of, 73 
retinal representation in, 79 
disturbances of, in brain tumor, 874 
treatment of, 892 

lumbar puncture, 892 
ventricular puncture, 

Keen's method of, 893 
hallucinations of, 174 
causes of, 174 
varieties of, 174 
with brain tumor, 388 
with epilepsy, 737 
with migraine, 707 
with multiple neuritis, 602, 604 
hyperesthesia of, with paresis, 479 
impaired near, 

with multiple neuritis, 605 
impairment of, Avith brain tumor, 874 

operation to prevent, 875 
with epilepsy, 732 
with multiple neuritis, 566 
with myelitis, 566 



Vision, line of, 36 
normal, 746 

peripheral limitation of, 169 
lesions causing, 169 
zone of, 166 
physiology of, 20 
single, antipathy to, 186 
stereoscopic, 182 
telescopic, 166 
Visual act, 

acuteness, 50 

reduction of, hysteria, 621 
apparatus, 20 
apraxia, 118 
aurae, 114 
cortex, 8, 59, 60, 75 

concrete conception, area of, 61 
connections and associations of, 63 
differences of, in man and lower ani- 
mals, 63 
evolution of, 69 
focal diseases of, 79 
histology of, 66 

layers of, 
Cajal's, 67 

Campbell's, 72, 73 
von Monakow's, 67 
image (binocular), 182 

in diplopia, 187 
limits and subdivisions of, 59 

Flechsig's, 60 
localization, 

histology in relation to, 66 
defects due to atrophy of cerebrum, 550 

in neurasthenia, due to eye-strain, 776 
disturbances, 

in astereognosis, 114 
in hemianesthesia, 114 
with acromegaly, 560 
with multiple neuritis, 602 
fibres, decussation of, 1 
field, boundaries of, 161 
changes, epileptic, 733 

with exophthalmic goitre, 841 
concentric contraction of, 
examination for, 624 
hysteric, 622 
types of, 626 
contraction, hysterical, 

nature of, 633 
defects of, 159 
disturbances of, 
hysteric, 621 

types of, 626 
with myelitis, 568 
extent for color, 162 
fatigue, 

diagnostic significance of, 684 et 

seq. 
types of, 681 et seq. 
hysterical, 634 
measurement of, 157 
neurasthenic anomalies, 675 et seq. 
oscillating (neurasthenia). 680 
peripheral zone of, 166 
sector-like defects in, 169 
shape of, 164 
fusion, 181 
impairment, 

with acromegaly, 562 
pragmatamnesia, 118 
psychology of, 57 
sensation (subjective). 174 
sense, (114 

anaesthesia of (complete), 
hysterical, 614 



988 



IKDEX 



Visual act, sense, anaesthesia of (incomplete), 
hysterical, 621 
signs 

of epilepsy, 731 
system, 

congenital absence of, 552 

Spiller's case of, 553 
Vitreous body, 

structure of, 31 

w 

Walking (see Gait), 

delayed, 949 

description of, 937 
Webster's syndrome, 

simulation of, by hysteria, 653 
Wernicke's disease, 452 

light test, 170 

symptom, 

in differentiation of anopsia, 85 
Wilbrand's light test, 170 
Winking, 

mechanism of, 349 
Word-blindness, 127 



Word-blindness, cortical, 128 
signs of, 128 

definition of, 128 

etiology of, 132 

lesions causing, 132 

mechanism of, 128 

pure, 128 

varieties of, 128 
Word-deafness, 

with brain tumor, 399 

with migraine, 725, 726 
Word-dumbness, 148 
Word-seeing, centre for, 128 
Wrist-drop, 

with multiple neuritis, 594, 599, 600 



Xanthopsia 

(yellow vision), 176 



Yellow vision, 176 



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